Turcot syndrome (TS) is a rare hereditary disorder clinically characterized by 
the occurrence of primary tumors of the colon and the central nervous system 
(CNS). Here we present the case of an 11-year-old boy with a synchronous 
clinical presentation of both glioblastoma multiforme (GBM) and colonic 
adenocarcinoma. A molecular genetic study revealed microsatellite instability in 
the DNA mismatch repair (MMR) gene. This patient ultimately survived for 13 
months after clinical presentation. Based on this case study, the synchronous 
presentation of glioblastoma multiforme and adenocarcinoma of the colon might 
suggest a shorter survival rate for patients with Turcot syndrome. A literature 
review complements this paper.