Introduction Solid Pseudopapillary Neoplasm (SPN) is a rare pancreatic neoplasm with low malignant potential and a relative indolent course. Complete resection of the SPN is curative for most cases and has a high survival rate. Recurrences, though rare, can still occur despite adequate resection. Pancreaticoduodenectomy is commonly performed to treat pancreatic head SPNs. In recent years, duodenum-preserving pancreatic head resection (DPPHR) has been reported as a less radical and acceptable alternative. Case presentation We are reporting a case of 26-year old female who presented with a 7 month history of epigastric pain and increasing abdominal girth. She was diagnosed by MRI to have a huge but resectable pancreatic head mass and subsequently underwent duodenum-preserving pancreatic head resection (DPPHR) with pancreaticojejunostomy. Histopathologic examination revealed a solid pseudopapillary tumor (SPN) with lymphovascular invasion and negative margins of resection. The patient underwent hepaticojejunostomy 5 months after resection for biliary stricture. Surveillance imaging revealed tumor recurrence warranting re-exploration for recurrence 3 years after the initial surgery. Intraoperative findings revealed the mass at the distal pancreatic remnant, requiring distal pancreatectomy and splenectomy. Discussion Solid pseudopapillary neoplasms are rare pancreatic neoplasms. Surgical resection of SPNs affords long term cure with good 5-year survival rates for localized tumors Despite the low malignant potential of SPNs, relapse after resection can still occur. Conclusion Complete local resection of the tumor is the treatment of choice in SPNs. DPPHR should be considered as an alternative in young patients with a localized SPN in the pancreatic head.