Extraspinal ependymomas are a rare type of glioma that may arise in th
e sacrococcygeal region, presenting as a pelvic mass in an infant or c
hild. Ependymoma presenting in the newborn period has not been describ
ed previously. Herein we describe a case of a newborn boy who presente
d with a perianal ependymoma, which was subsequently found to have pre
sacral extension. The major diagnostic challenge this case presented w
as to rub out the alternative diagnoses of sacrococcygeal teratoma or
a developmental malformation/heterotopia.