Teratomas are rare tumors of childhood with an incidence between 1:20.000 to 1:40.000 births. 5% of those teratomas are located in the head and neck region. Without treatment 80% of the little patients with oropharyngeal teratomas die in the postpartum period because of airway obstruction. The treatment of oropharyngeal teratoma, especially when extended to intracranial cavity, remains very difficult. Establishment of a secure airway is the most important step after delivery. The complete surgical resection has to be performed without creating significant growth impairment. A case of a female patient with an intracranial extended oropharyngeal teratoma is presented. A lateral transmandibular approach to the scull base with temporal exarticulation of the mandible was used for final tumor resection. 6 years after surgery there were no neurological deficits but hemimandibular microsomia treated successfully with distraction ostegenesis. The technical aspects of the surgical technique used are demonstrated and treatment modalities of oropharyngeal teratomas are described. Keywords: Teratom, Kleinkind, Schädelbasis, transmandibulärer Zugang, Distraktionsosteogenese, Unterkiefer