Abstract: Objective：To study the clinicopathological features, immunophenotype and differential diagnosis of rhabdomyosarcoma(RMS) in head and neck. Methods：The clinical, pathologic and immunohistochemical features were evaluated in 11 cases of RMS in head and neck. Results：11 cases were retrieved from the pathological archive of this hospital. 8 cases were male, and 3 cases female, with their ages ranging from 2 to 51 years (median, 25 years). Typical clinical presentation was characteried by pain or painless masses located in head and neck. Histological subtypes：8 cases were embryonal rhabdomyosarcoma(ERMS)，3 cases alveolar rhabdomyosarcoma(ARMS). Immunohistochemically, all cases were positive for desmin and vimentin. The positive staining rates for MyoD1 and myogenin were 45% and 55%, respectively. 2 cases were positive for Syn. The remaining antibodies(CK, LCA, CD99)were all negative. Follow-up information was available in 8 cases, ranging from 6 months to 5 years, which revealed that 2 patients died of the disease and 4 patients developed local recurrences, and 2 patients had metastasis. Conclusions：RMS in head and neck is a rare neoplasm, and the clinical presentations are not typical. The diagnosis can be established by hispathological and immunohistochemical examination.