Abstract Introduction Solid pseudopapillary neoplasm (SPPN) was first characterized by Virginia Frantz in 1959. The duodenum-preserving pancreatic head resection (DPPHR) has been described as treatment for low-grade malignant tumors of the head of the pancreas including 8 cases of SPPN Presentation Of Case: A 16-year-old white female patient presented with abdominal pain and dyspepsia. Computed tomography scan of abdomen showed a 10×9x10cm lesion on the pancreatic head. After radiological diagnosis of SPPN the patient was submitted to DPPHR. Resection was achieved with clear margins. Immunohistochemical study demonstrated positivity for progesterone receptor, β-catenin, cytoplasmic paranuclear dot-like CD99, negativity for chromogranin and S100 protein and Ki 67 index of 1%. Discussion A large encapsulated pancreatic mass with well-defined borders that contains areas of calcifications and intratumoral hemorrhage on CT scan in a young female is virtually diagnostic of an SPPN. A particular dot-like intracytoplasmic expression of CD99 appears to be highly unique for SPPN Conclusion DPPHR should be considered in cases of SPPN in the pancreas head if there is no compromise with oncologic radicality.