Multiple endocrine neoplasia type 1 (MEN1) is an inherited predisposition to tumors of the parathyroid glands, anterior pituitary, and pancreatic islet cells. In this review, we discuss the clinical case of a 45-year-old woman with MEN1 that was presented at the 2015 North American Neuroendocrine Tumor Society Symposium. In our review of this patient's complicated clinical course and subsequent operative management, we highlight controversies in the diagnosis and management of pancreatic neuroendocrine tumors in MEN1. In particular, this case illustrates the lack of consensus regarding the optimal biochemical and radiologic screening for pancreatic neuroendocrine tumors and absence of guidelines about the appropriate surgical approach for treatment. We review these controversies and discuss possible approaches to management.