Synchronous pancreatic neuroendocrine tumor (pNET) and pancreatic cyst are very rare. Herein, we report a case of pNET combined with a pancreatic cyst in a 49-year-old female. The patient first presented with a complaint of persistent pain in the right upper abdomen. As a follow-up, CT scan was performed that showed a solid pNET of 2-cm in diameter and a pancreatic cyst of 5-cm in the tail. Then, the subsequent distal pancreatomy with splenectomy was performed. The tumor histologically diagnosed with a cystic pancreatic neuroendocrine tumor. Therefore, it is critical to get a deep insight into this seldom tumor. Surgery remains the standard option of treatment for pNET.