This case report discusses a rare occurrence of primary sciatic leiomyosarcoma in a patient with type 1 neurofibromatosis. The patient had a gradually enlarging lump in her right thigh, which was diagnosed as a leiomyosarcoma after surgery. The association between neurofibromatosis type 1 (NF1) and leiomyosarcoma has not been well-established. However, smooth muscle tumors in NF1 patients are often multitudinous, and careful follow-up is crucial due to the potential development of leiomyosarcomas. The case report highlights the diagnostic and management challenges presented by this rare occurrence. The patient underwent surgical resection of the tumor(14×11cm), including a portion of the proximal and distal sciatic nerve, achieving an R0 margin,followed by radiation therapy (60 Gy in 30 fractions). The postoperative period was uneventful, with no recurrence or metastasis observed during the six-month follow-up period.
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