ACTA FACULTATIS MEDICAE NAISSENSISUDC: 616.831-006Teratomas constitute a group of nongerminomatous germ cell tumors that are composed of an admixture of different tissue types representative of ectoderm, endoderm and mesoderm. Intracranial teratomas are rare, comprising approximately 0.5% of all intracranial tumors. They preferentially involve the midline structures, and occur primarily in children.We presented an unusual case of mature intracranial teratoma with extracranial extension in a 24-year-old man who complained of headache, nausea and vomiting. The intracranial expansive lesion was localized in the left frontotemporobasal region extending into the ipsilateral orbit. The patient underwent total resection of the tumor. No adjuvant treatment was given. On gross examination, the resected tumor was lobulated, containing heterogeneous solid and cystic components. Histologic investigation revealed the presence of various fully differentiated tissues representative of the three germ cell layers, including adipose tissue, cartilage, bone, striated muscle bundles, brain tissue, respiratory epithelium and glandular structures. The diagnosis of mature teratoma was established. Follow-up at four years did not show any evidence of recurrence.The presented case is an unusual example of mature teratoma with regards to the age of the patient and, especially, to the location of the tumor, both intraand extracranially. It is critical to recognize this rare entity by extensive sampling to rule out the presence of immature elements which may constitute only a minor part of the tumor.