Solid pseudopapillary neoplasms (SPNs) are exceedingly rare type of exocrine pancreatic malignancy, representing only 0.9%-2.7% of all exocrine pancreatic malignancies. They predominantly affect young women and unlike other pancreatic malignancies, they have excellent prognoses with 5-year survival following surgical resection approaching 97%. Given the rarity of the disease, little is known about their histopathogenesis as they do not harbor similar genetic mutational abnormalities like other pancreatic tumors. We describe a rare case of SPN in a young female who was found to have the rare diagnosis during the work up for deranged liver function tests.

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