INTRODUCTION: Small cell pancreatic neuroendocrine carcinoma (P-NEC) is in a group of malignancies described as Gastroenteropancreatic (GEP) NEC, and is an extremely rare neoplasm. We present a case of P-NEC with confounding findings of multiple ovarian and pancreatic masses. CASE DESCRIPTION/METHODS: A 33-year-old European female with history of ovarian cysts (∼4 cm × 5 cm) presented with a ten-day history of lower abdominal/pelvic pain with new onset ascites and weight gain. No family history of GI or gynecologic malignancy. A CT (Figure 1a,b) of her abdomen and pelvis showed ascites, multiple pancreatic hypodense lesions and enlarged cystic ovaries (10 × 11 cm). Subsequent MRI pancreatic protocol (Figure 1c,d), and an endoscopic ultrasound (EUS-FNB) was performed. MRI showed diffusely abnormal pancreas with multiple focal solid masses with T2 hyper intense, T1 hypo intense, and hypo-enhancing lesions on early post contrast phases with no pancreatic ductal dilation or cystic component. EUS confirmed multiple hypodense pancreatic lesions but the findings were not consistent with primary pancreatic malignancy. Cytopathology of gastric nodules (Figure 2) and the pancreatic FNB (Figure 3) showed small cell NEC with a Ki-67 proliferative index (PI) of 43%. Pathology showed infiltrative process with small blue cells and scant cytoplasm. The IHC was positive for synaptophysin, chromogranin, CD56, weakly positive for CAM5.2 and AE1/AE3, negative for TTF-1, PAX8, CD45, CDX2, ER, and inhibin. The diagnosis was pancreatic small cell NEC. The enlarged ovarian cysts were determined to be an incidental finding, and not a primary source of the malignancy. DISCUSSION: Generally, the well differentiated pancreatic neuroendocrine tumors (NET) are hormone secreting; however, the GEP NEC are a highly aggressive poorly differentiated carcinoma and designated as grade 3 tumors. Grading is based on the Ki-67 PI and mitotic count/index (Table 1). Our patient had GEP NEC given her Ki-67 PI was 43%. Chemotherapy and/or surgical resection are the two treatment options, but by the time of late diagnosis the goal of the treatment is generally palliative. Our case emphasizes the importance of pursuing accurate pathological diagnosis, role of EUS in management of pancreatic neoplasms and eliminating the confounding factors (eg ovarian cysts/masses).