Abstract Primary rhabdomyosarcoma of salivary glands is an extremely rare neoplasm, mostly seen in children. A newly described subtype of rhabdomyosarcoma, sclerosing rhabdomyosarcoma, has not yet been reported in this location. We report on a parotid gland tumor characterized by infiltrative growth of primitive type of neoplastic cells showing strong and diffuse nuclear positivity for MyoD1 and myogenin and by prominent hyalinized/chondroid matrix with some myxoid foci. The tumor recurred several times, and in recurrent tumors, differentiation into strap myoid cells appeared. There were no distant metastases during the 5-year follow-up. Sclerosing rhabdomyosarcoma may cause differential diagnostic problems because it could be confounded for osteosarcoma, chondrosarcoma, and some other types of sarcoma, and as in our case, for myxofibrosarcoma and myoepithelial carcinoma. Its location in the head and neck is of special interest because 6 of 14 previously described adult cases of sclerosing rhabdomyosarcoma and 7 of 18 pediatric cases also occurred in this region. To our knowledge, this is the first reported case of primary sclerosing rhabdomyosarcoma of the parotid gland.