For three patients, previously diagnosed with an alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma and undifferentiated pleiomorphic sarcoma (UPS) of the jaw, respectively, their diagnosis was revised to spindle cell/sclerosing rhabdomyosarcoma based on a MYOD1 p.Leu122Arg mutation in two patients, and an FUS–TFCP2 fusion in the presumed UPS patient. The gene discussed is MYOD1; the disease is alveolar rhabdomyosarcoma.