Abstract:This study reports on a non familial case of Turcot's syndrome in a 40‐year‐old man with an astrocytoma (grade II) in the pineal region, colonic polyposis and four adenomas in the gastric antrum. In previous reports, most patients with Turcot's syndrome revealed symptoms until early in the fourth decade of their lives. In most cases, a complete resection of the brain tumors were impossible, and/or colonic polyps had already developed malignant transformation at the time of diagnosis, which led to a poor prognosis. In our case, however, the brain tumor was completely resected and a detailed histological analysis of the polyps in the colectomy specimen revealed no malignant transformation. A good prognosis, therefore, could be expected with a careful follow‐up. In this paper, we compare our case with 50 previous cases of Turcot's syndrome histopathologically confirmed and reported in the literature, with special reference to their gastrointestinal manifesta