A 75-year-old man without underlying disease was referred to our department presenting with abdominal distension. Laboratory tests were unremarkable except for a significant elevation of carbohydrate antigen 19-9. An abdominal computed tomography (CT) scan demonstrated an approximately 8 cm-sized mass with a necrotic portion in the pancreatic tail and multiple hypodense masses involving liver, spleen, and adrenal gland with massive ascites (Fig. 1A and 1B). A chest CT scan showed no evidence of pulmonary tumor. A positron emission tomography scan revealed intense hypermetabolic uptake in the pancreatic tail, liver, spleen, both adrenal glands, and peritoneum (Fig. 1C). Endoscopic ultrasonography (EUS) showed an ill-defined heterogeneous mass in the pancreatic tail with peripancreatic and perihilar lymph node enlargement. EUS-guided fine needle biopsy (FNB) was performed with 22-G core needle on the pancreatic mass. On histology, small round cells with nuclear molding and crush artifacts (Fig. 2A). Immunohistochemical (IHC) staining was negative for thyroid transcription factor (TTF-1), leucocyte common antigen, and positive for CD56, which was compatible with primary pancreatic small cell carcinoma (PPSCC) (Fig. 2B and 2C). The patient refused palliative chemotherapy and expired 3 weeks later.