Gorlin-Goltz Syndrome and Situs Inversus Totalis coincidental occurrence is unlikely. But both entities together were found is the rarest bestowal. A search conducted on the PubMed revealed that there is no such unique occurrence in the world literature. Evaluation and treatment of NBCCS requires a multidisciplinary approach involving Dentists, Maxillofacial Surgeons, Dermatologists, Radiologist and Neurologists. This poster will throw light on clinical as well as radiological features of this rarest combination to solve a diagnostic enigma. There is the intriguing possibility that these two clinical phenotypes are histogenetically linked to PTCH/hedgehog signaling. The hedgehog (Hh) signaling pathway plays an important role both in embryonic development and adult stem cell function. Dysregulation of the pathway causes birth defects and cancer. NBCCS is known to be caused by mutations in the PTCH1, the receptor of hedgehog. PTCH1 is localized to primary cilia and mediates key steps in the transduction of the hedgehog signal. It has been suggested that ciliary dysfunction via dysregulation of the hedgehog pathway is the underlying cause of situs-inversus. Introduction: Unique occurrence of Gorlin-Goltz Syndrome with Situs Inversus Totalis is for the first time reported in the world literature by Dr. Monika Gupta in 2011. Gorlin-Goltz syndrome was firstly reported by Jarish and White in 1894. Situs inversus totalis means complete mirror image of thoracic and abdominal organs was firstly described by Matthew Baillie in 18th century. Gorlin-Goltz Syndrome and Situs Inversus Totalis coincidental occurrence is unlikely. But both entities together were found is the rarest bestowal. A search conducted on the PubMed revealed that there is no such unique occurrence in the world literature. Evaluation and treatment of NBCCS requires a multidisciplinary approach involving Dentists, Maxillofacial Surgeons, Dermatologists, Radiologist and Neurologists. This poster will throw light on clinical as well as radiological features of this rarest combination to solve a diagnostic enigma. There is the intriguing possibility that these two clinical phenotypes are histogenetically linked to PTCH/hedgehog signaling. The hedgehog (Hh) signaling pathway plays an important role both in embryonic development and adult stem cell function. Dysregulation of the pathway causes birth defects and cancer. NBCCS is known to be caused by mutations in the PTCH1, the receptor of hedgehog. PTCH1 is localized to primary cilia and mediates key steps in the transduction of the hedgehog signal (1-3). It has been suggested that ciliary dysfunction via dysregulation of the hedgehog pathway is the underlying cause of situs-inversus (4). Case presentation: A 13-year-old girl reported to the department of oral and Maxillafacial surgery. She complained of painless swelling on the lower left side of her face. The swelling was measured 4 × 3 cm, firm and non-tender. Frontal bossing and a wide nasal bridge were also noted. Dermatological examination revealed palmar and plantar pits and keratosis were present. Psychiatric consultation showed intellectual deficit, and ophthalmological examination revealed hypertelorism. Orthopantomography showed multiple cystic lesions in the upper and lower jaws, which were associated with unerupted permanent teeth displaced from their normal positions. Axial computerized tomography showed calcification of the falx cerebri along the venous sinuses and small venous channels. Lateral views of the skull revealed bridging of the sella turcica. A chest radiograph showed bifid fourth and fifth ribs on the left side and dextrocardia, with the heart apex to the right side. Ultrasound examination of the abdomen confirmed complete situs inversus; the liver was on the left, the spleen on the right side, and the stomach was transposed. Discussion and Conclusion: A search conducted on the PubMed revealed that there is no such unique occurrence in the world literature. However, the rarity of these two conditions (NBCCS incidence: 1 in 57,000; situs inversus incidence: 1 in 10,000) suggests that coincidental occurrence is unlikely. Thus, There is the intriguing possibility that these two clinical phenotypes are histogenetically linked to PTCH/hedgehog signaling. The hedgehog (Hh) signaling pathway plays an important role both in embryonic development and adult stem cell function. Dysregulation of the pathway causes birth defects and cancer. NBCCS is known to be caused by mutations in the PTCH1, the receptor of hedgehog. PTCH1 is localized to primary cilia and mediates key steps in the transduction of the hedgehog signal (1-3). It has been suggested that ciliary dysfunction via dysregulation of the hedgehog pathway is the underlying cause of situs-inversus. Palabras clave: Situs Inversus Totalis, Gorlin-Goltz Syndrome, PTCH1 Mutation, Ciliary Dysfunction