Solid pseudopapillary neoplasm of the pancreas is a rare tumor of the pancreas often detected initially on imaging. Of uncertain histogenesis, it has a low-grade malignant potential with excellent post-surgical curative rates and rare metastasis. Despite advances in imaging, pseudocysts and other cystic neoplasms feature in the differential diagnosis. Pathological and/or cytological evaluation remains the gold standard in reaching a definitive diagnosis. On morphology alone, other primary pancreatic tumors and metastatic tumors pose a diagnostic challenge. Recent advances in immunohistochemical characterization have made the histopathologic diagnosis more specific and, in turn, shed light on the likely histogenesis of this rare tumor. We report a case of solid pseudopapillary neoplasm of the pancreas that was suspected on radiology and diagnosed intraoperatively on imprint cytology guiding definitive surgery. The diagnostic dilemmas are reviewed.
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