Introduction: The pancreatic endocrine tumors are rare. Insulinoma is the most common pancreatic endocrine neoplasm.30% of pancreatic tumors in MEN type I are beta cell tumors.

Case Report: The presented case is a 23 year old male with a family history of MEN I syndrome .The patient presented with whipple triad. Radiologic studies revealed two small and round masses in body and tail of pancreas. With clinical diagnosis of pancreatic tumor the patient underwent laparatomy, distal pancreatectomy and splenectomy.

Conclusion: According to macroscopic, microscopic, immunostaining findings, clinical and laboratory data the final diagnosis was insulinoma.