INTRODUCTIONNeuroendocrine neoplasms (NENs) represent a heterogeneous group of tumors, which are categorized into either low-grade indolent tumors, referred to as neuroendocrine tumors (NETs), or high-grade aggressive carcinomas, known as neuroendocrine carcinomas (NECs)1,2. Markers of neuroendocrine differentiation include chromogranin A, chromogranin B, and synaptophysin1,2. NENs may be functional, synthesizing excess bioactive hormones which leads to various clinical manifestations such as abdominal pain, diarrhea, hyperglycemia, and skin manifestations such as acanthosis nigricans2. Neuroendocrine cells are widely dispersed in the body; consequently, NENs can manifest in nearly any organ. However, they are rarely found in the pancreas. Among this small portion of pancreatic NENs, those that secrete ACTH and cause Cushing’s Syndrome (CS) are even rarer. To our knowledge, only 13 cases have been documented3-16. In this article, we outline the clinical progression and treatment of this tumor, contributing to the limited body of literature that exists describing this tumor.