A rare case of adult medullomyoblastoma with unique location presented. The patient was admitted to our clinic with the history of progressive balance disorder, nausea and vomiting. Disequilibrium had started 2 months earlier. The patient underwent a craniotomy and histopathologic examination revealed a biphasic tumor. Few definite strap cells with striation were identified in the tumor. The diagnosis was medullomyoblastoma. Histopathological evaluation is crucial for diagnosis of MMB. Tumor resection is the first step of treatment.

Keywords: Medullomyoblastoma, posterior fossa tumors, primitive neuroectodermal tumors, cranial surgery