Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome associated with the development of many endocrine tumors, involving mainly pituitary, parathyroids, pancreas, although a proliferative state interests all neuroendocrine system. MEN1 pancreatic neuroendocrine tumors (pNETs) are multiples and can secrete different hormones. The therapeutic approach is based on surgery which usually is followed by tumor relapse or persistence unless to be highly aggressive. Biotherapy with somatostatin analogs and dopamine agonists could be of great benefit to manage these patients without altering their life quality. We report a case of a 36-year-old MEN1 man affected with multicentric pNETs associated with insulinoma syndrome. Therapy with symptomatic agents (diazoxide), as well as biotherapy (lanreotide, cabergoline) was started. At 6-month follow-up, symptomatic agents were stopped and disease control was only based on lanreotide plus cabergoline. This combined biotherapy was able to control endocrine syndromes and tumor growth. Subsequently, a safer and selective surgical intervention on pNETs was performed. An excellent response to therapy with lanreotide autogel and cabergoline has been observed in a MEN1 patient with pNETs associated with insulinoma syndrome. The potential synergistic effects of lanreotide autogel and cabergoline on insulin-secreting neuroendocrine tumors are discussed.