Objective: To improve the understanding of the diagnosis and treatment of pancreatic primitive neuroectodermal tumor (PNET). Methods: To analyze case reports of pancreatic PNET in our hospital and to describe the diagnosis and treatment of pancreatic PNET. Results: Pancreatic PNET is extremely rare. In this case, radiation therapy and 6 courses of chemotherapy were administered after resection of pancreatic hook tumor with preservation of duodenum, and there was no recurrence at 8 months of follow-up. The clinical symptoms of pancreatic PNET were nonspecific, mainly obstructive jaundice and abdominal pain. Diagnostically, in addition to a small round cell malignant tumor and neurodivergent structures under light microscopy, immunohistochemical results showed CD99 (+) of the pancreatic head hooks against the specific MIC2 gene expressing protein P30/32, and cytogenetics showed chromosomal abnormalities of t(11;22)(q24;q12). It is consistent with the diagnosis of primitive neuroectodermal tumor/Ewing’s sarcoma (PNET/Ewing’s sarcoma), which is highly malignant, and surgical treatment, early chemotherapy, and close follow-up may be the main means to improve survival, with a generally poor long-term prognosis. Conclusion: Clinical and pathologists should be aware of the presence of pancreatic PNET, improve clinical and pathologic diagnosis, and treat it early and aggressively with a view to improving prognosis.