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Solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is a rare neoplasm of the pancreas accounting for only 0.17–2.7% of all pancreatic neoplasms, often detected initially on imaging. Despite advances in imaging, pseudocysts and other cystic neoplasms feature in the differential diagnosis. We report a case of solid pseudopapillary epithelial neoplasm in a young female who presented with a progressively increasing abdominal lump in whom the diagnosis was considered based on imaging studies. She underwent exploratory laparotomy, pancreaticoduodenectomy, pancreaticojejunostomy, hepaticojejunostomy, gastrojejunostomy, and made an uneventful recovery in the postoperative period. Histopathological examination and immunohistochemistry confirmed the diagnosis. This case highlights the need for a high index of suspicion and timely surgical intervention for optimal outcomes.