Turcot syndrome is a rare inherited disorder characterized by the association of colon and central nervous system neuroepithelial tumors. Here, we present a patient with Turcot Syndrome having glioblastoma together with colon adenocarcinoma. The patient, 47 year-old male, was referred to our department for cranial radiotherapy after gross-total resection of primary brain tumor in October 2003. During clinical follow-up in October 2004 a right colon mass was determined and right hemicolectomy was performed. Six courses of adjuvant chemotherapy were applied after resection. The patient is still under follow-up without any recurrences or metastasis. He has also first-degree relatives with colon cancer in his family's history. It has been declared that patients with Turcot Syndrome who have high-grade glial tumor, survive longer than other brain tumor patients. With some certain exceptions, all these patients were young. There are few patients who were diagnosed of glioblastoma at the fifth decade and had long survival period. Present case is elderly diagnosed with Turcot syndrome and has long survival for glioblastoma, therefore, it is a rare clinical manifestation of Turcot Syndrome.