Head and neck rhabdomyosarcoma (HNRMS) is exceedingly rare and poorly documented; it is a rare malignant soft tissue neoplasm comprised of cells derived from the primitive mesenchyme. About 35% of RMS arises in the head and neck, are classified as parameningeal and non-parameningeal forms. These are the most common soft tissue sarcoma of the children, adolescents and young adults. Their etiopathogenesis and its molecular relevance have been emphasized. The first line of treatment is radical excision and this is usually supplemented by radiotherapy. It is believed that adjunct combination chemotherapy may greatly improve the prognosis. Inadequately treated tumours grow in an infiltrative manner and recur in a high percentage of cases. Bone does not constitute an effective barrier to the growth of the tumour and bone invasion is a frequent finding in head and neck rhabdomyosarcomas.