[Objective] To explore the clinical diagnostic and therapeutic characteristics of peripheral primitive neuroectodermal tumor (PNET). [Methods] A retrospective analysis was conducted on the clinicopathological data of a patient diagnosed with seminal vesicle PNET treated in our hospital.Relevant literature was retrieved in PubMed, Web of Science, and CNKI.The clinical features, pathological results, and treatment methods were reviewed. [Results] Including this case, a total of 6 cases were collected.This case underwent a radical cystectomy with transabdominal approach, bilateral seminal vesicle resection, pelvic tumor resection, and ileal bladder surgery.Postoperative pathology confirmed PNET.During the follow-up of 32 months, no local recurrence or distant metastasis recurred. [Conclusion] Seminal vesicle PNET is a rare tumor with high malignancy, rapid progression, and poor prognosis.The current diagnosis mainly relies on pathological biopsy and immunohistochemistry.Surgery is an effective treatment method.