Abstract
Renal LGFMS is an extremely rare tumor. So far, only four cases have been reported in literature. LGFMS are indolent tumor with distinctive histopathological features with potential for late recurrences and metastasis. The diagnosis is made on histopathological examination and supported by immunohistochemical analysis. A 35-year-old young male was presented with flank pain and lump and diagnosed as low-grade fibromyxoid sarcoma after surgery on immunohistochemical analysis. This is the fifth case of renal LGFMS and we presented the review of literature. Radical surgery remains the primary treatment modality. Long follow-up is mandatory.