Congenital cerebellar medulloblastoma is extremely rare. Reported here is a female infant who presented her first abnormal clinical manifestations on the sixth day of life and who was subsequently found to have medulloblastoma at operation. Electron microscopy revealed glial filaments and short cytoplasmic projections in some neoplastic cells and the presence of junctional complexes between neoplastic cells with and without glial filaments, which suggests the astrocytic differentiation of the tumor. Including our own case, there have been only 21 reported cases of congenital cerebellar medulloblastoma. A strong female preponderance and some familial occurrence are noted in congenital cerebellar medulloblastoma.