Sclerosing rhabdomyosarcoma (SRMS) is a very rare subtype of rhabdomyosarcoma (RMS) and can lead to diagnostic difficulties especially if one is not advised of this variant. This entity was first described in 2000 by Mentzel and Katenkamp.Morphologically, it is characterized by an abundant hyalinized stroma that may erroneously lead to the diagnosis of extraskeletal chondrosarcoma, sometimes this stroma simulates a primary osteoid or vascular tumor. Thus, it can lead to confusion with osteosarcoma or angiosarcoma. We report here a case of SRMS mimicking a sclerosing epithelioid fibrosarcoma appearing in calves in a 55-year-old man. The tumor cells were arranged in nests, cords with sometimes a pseudovascular features. Immunostaining showed that the tumor was positive for Desmin, SMA and MyoD1, focally positive for myogenin and negative for CK, P63, EMA, S-100, H-caldesmon, CD34, CD31. Based on morphological discovery and immunostaining, he was diagnosed as an SRMS. This is the first case of SRMS to show a strong and diffuse α- SMA highlighting the risk of misdiagnosis as leiomyosarcoma.