Peripheral primitive neuroectodermal tumor (pPNET) is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usualy occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed smal round-celltumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes.