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Multiple endocrine neoplasia type 1 (MEN1) is a rare, hereditary, autosomal dominant disease. It is characterized by the presence of cell hyperplasia or tumors of the endocrine system: parathyroid, duodenopancreatic, thymic, and bronchial neuroendocrine tumors (NETs), and pituitary and adrenal adenomas. Duodenopancreatic involvement is common and accounts for 60% of the causes of death from MEN1. Duodenopancreatic NETs can be functional or non-functional, benign or malignant. The most common functional duodenopancreatic NETs are gastrinomas and insulinomas. Patients with MEN1 require multidisciplinary management (gastroenterologist, endocrinologist, pathologist, radiologist, nuclear physician, surgeon) in an expert center and lifelong follow-up. Failure to diagnose MEN1 is a missed opportunity for patients and their families. This review focuses on duodenopancreatic NETs in MEN1, specifically their diagnostic and therapeutic management.