Turcot syndrome is an association of primary neuroepithelial tumors of the central nervous system with adenomatous polyposis coli. It is a genetic disorder, with two forms; In type I, glioblastomas are usually associated with hereditary nonpolyposis colorectal cancer (HNPC or Lynch). In Type II, medulloblastomas are often associated with familial adenomatous polyposis coli (classical or attenuated). This patient had a medulloblastoma at seven years of age, then 20 years later developed a meningioma which recurred several times. At 36 years old he presented with anemia after digestive bleeding, and an adenomatous polyposis coli with high grade dysplasia was found at colonoscopy. As far as we know, this is the first case of Turcot syndrome described in our country.