Introduction : Solid pseudopapillary neoplasia of the pancreas is an extremely rare epithelial tumor of low malignant potential that primarily affects young women.

Methods : We report a case of 19-year-old woman with huge pancreatic head cyst presented with mild jaundice, and right upper quadrant pain. CT scan suggested a 13cm diameter, multi-cystic mass with solid component and enlargement of regional lymph nodes.

Results : Preoperatively we diagnoses this patient with huge pancreatic head cyst with malignant potential. Due to the inavailability of ERCP, EUS and FNA, we proceed with surgical resection. She underwent pancreaticoduodenectomy, with post-surgical biopsy confirmed a solid pseudopapillary neoplasia, localized to the pancreas. The post-operative course went uneventfully, and was discharged on post-operative day 10. No adjuvant therapy was given to this patient, and she remained asymptomatic and showed no signs of disease after four months follow-up.

Conclusions : It is important to differentiate a solid pseudopapillary neoplasia tumor from other pancreatic cystic neoplasms, because this type is amenable to cure after complete surgical resection, even in cases with capsular invasion, unlike malignant tumors of the pancreas