Rhabdomyosarcoma (RMS) is a common childhood cancer, constituting more than 50% of all soft tissue sarcoma, but it is an uncommon neoplasm in adult. We reported a case of 45-year-old male patient presented with a huge swelling in his left thigh. Magnetic resonance imaging of thigh revealed soft tissue mass involving the deep muscular compartment. Core biopsy and immunohistochemistry confirmed it as an embryonal RMS. The tumor showed a complete response after three cycles of neoadjuvant chemotherapy (CT) and then was treated with three more cycle of CT followed by adjuvant radiotherapy. This case is being reported on account of its rarity at this age and nonsurgical treatment. Pertinent literature is being reviewed.
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