Background: Ewing sarcomas are among the most frequent malignant bone tumors among children and young adults. Extraskeletal Ewing sarcomas are a rarity, even more so within the Head&Neck-Region.. Worldwide there are only a handful of cases published. Methods: A 20 years old male patient was presented to our clinic by a colleague in private practice. He had removed a pea-sized pediculated tumor from the anterior base of the mouth which he considered to be a granuloma. The histopathological analysis unveiled a Ewing sarcoma which was validated by two further reference labs. We proceded to perform an exended excision around the basis of the former tumor pedicule. Within the specimen no remains of the sarcoma could be found. The consecutive radiological diagnostics - MRI & 18F FDG-PET/CT - did not reveal any remaining tumor as well as no suspicious structures for metastasic disease. The interdisciplinary tumor conference agreed upon an adjuvant radio-chemotherapy. This recommendation, however, was rejected by the patient. For this reason consecutive clinical and radiological (MRI and PET/CT) follow-ups were arranged with the patient on a short-time interval basis. For 60 months the patient has been involved in follow-up surveillance. Up tll now there has been no sign of relapse or metastasis. Conclusion: For the treatment of Ewing sarcomas within children and young adults an established S1-Guideline (AWMF) exists that aim primarily at the treatment of skeletal manifestations. For the treatment of Ewing sarcomas of soft tissue, especially within the Head&Neck-region, the guideline presents us with a challenge regarding borders of resection. Despite being a very rare disease specific therapy guidelines should be available also fort he Head&Neck-region. Keywords: Extra-skeletal ewing sarcoma, intra oral sarcoma, sarcoma treatment