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Four tumours including neuroendocrine tumour of the ileum. Gastroenteropancreatic neuroendocrine tumours are associated with development of further primary tumours. Certain conditions give an inherited predisposition to developing neoplasia. To the best of our knowledge, this is the first paper to report a patient with neuroendocrine tumour and three other tumours: a further bowel cancer, a brain tumour and a skin cancer. We report a case of a 42-year-old Caucasian man presenting with clinical features of intestinal obstruction who on histopathology of surgical specimen was found to have two distinct tumours of the bowel: a colonic adenocarcinoma at the splenic flexure and a well-differentiated neuroendocrine tumour of the terminal ileum. His history included a basal cell carcinoma and a benign brain tumour. He had extensive family history of neoplasia suggesting an inherited predisposition. Our case demonstrates the importance of investigating patients with known neuroendocrine tumours for further malignancy and suggests that patients with multiple primary malignancies should be referred for genetic testing.