Patients with Turcot’s syndrome typically have a defect in the adenomatous polyposis coli (APC) gene and/or a mutation in DNA mismatch repair (MMR) genes predisposing them to the development of multiple colorectal adenomas, colorectal adenocarcinoma, and primary brain tumors (Itoh et al., 1993; Melean et al., 2004). This evidence concerns the gene APC and primary brain neoplasm.