A rare tumour occurring in the cerebellar midline and consisting of a mixture of medulloblastoma and mature and immature striated muscle is usually referred to as "medullomyoblastoma". The origin and nature of medullomyoblastoma are controversial. In this report, we describe the histological, and immunohistochemical features of medullomyoblastoma occurring in the cerebellar vermis of an 11-year-old boy.