Rhabdomyosarcoma is the most common soft tissue malignant tumor arising in children and young adults and it is rare in people older than 45 years. Many studies o f this tumor have been providing improvement of the prognosis. However, importance of the immunohistochemical expression of some proteins needs to be clarified. Therefore, the objectives o f this study were to analyze the clinicopathological and immunohistochemical features o f head and neck rhabdomyosarcomas and its relationship with the prognosis o f the patients. From 1954 to 2002, twenty-nine patients with head and neck rhabdomyosarcoma treated in the Department of Head and Neck Surgery and Otorhinolaryngology, CancerHospital AC Camargo, Sao Paulo, Brazil, were selected for this study. Clinicai data were obtained from the medicai records, and the histological slides were reviewed and the tumors were classified according to Enzinger and Weiss (2001). Immunohistochemical analysis was performed using primary antibodies anti vimentin, desmin, myogenin, MyoD1 and AE1/AE3 for diagnostic and p53, PCNA, Ki67, C-erbB2, FAS and CDK4 for prognostic significance by immunoperoxidase technique. The tumors were more frequent in the first two decades of life (mean age of 14.3 years) with a discreet male predilection (58.6%). The extraoral region was the most common location (62.1%) with prevalence on the nose. Intraoral involvement was observed in 37.9% of the cases, being the buccal mucosa the most frequent site. The predominant clinicai stage was T2b (9 cases, 31 %).Most patients did not show metastasis at the moment o f the diagnosis, however 1 O patients presented regional metastasis and two displayed distant metastasis. Microscopically, 18 cases (62.1%) were classified as embryonal, 10 (34.5%) as alveolar and 1 (3.4%) as pleomorphic. Immunohistochemical analysis for diagnosis showed positivity for desmin in