Multiple endocrine neoplasia 1 (MEN 1) is a rare genetic disorder classically characterized by a predisposition to tumors of the parathyroid glands, anterior pituitary gland, and pancreatic islet cells (1). In addition to exhibiting these characteristic tumors, MEN 1 patients also have an increased propensity for other tumors such as carcinoids, adrenal adenomas, angiofibromas, and lipomas (1, 2, 3). Although MEN 1 is rare, with a prevalence of approximately 2 per 100,000 people, recognition of this syndrome is extremely important for both patient treatment and evaluation of family members (1, 4). The tumors of MEN 1 are usually benign; however, malignancy of some carcinoid, islet cell, and gastrointestinal tract tumors can cause mortality (5, 6, 7, 8). Diagnosis of MEN 1 is usually made by a combination of history and physical examination, biochemical serum testing, and various imaging modalities (9, 10). We present a classic case of MEN 1 with unique presentation and diagnosis using predominantly nuclear imaging in order to emphasize the role of nuclear imaging in diagnosing and treating MEN 1.