Rhabdomyosarcoma (RMS) is a malignant tumor of soft tissues, more common in childhood, mainly located in the head and neck. It presents varied clinical and biological behavior and requires individualized management. Aim To describe information on patients with head and neck RMS diagnosed and treated in a hospital, and to compare them to results in the literature. Study design: Descriptive and retrospective. Materials and Methods A retrospective analysis of data from 24 patients with head and neck rhabdomyosarcoma diagnosed and treated in a hospital from 1994 to 2008. Results The mean age was 7.79 years. According to gender, 54.17 % were males and 45.83 % were female. All patients underwent chemotherapy (CT), 62.5% of them also underwent radiotherapy (RT) and 16.67% were submitted to surgery. Of the 24 patients, 8 (33.3%) died, 6 (25%) were found free of neoplasia and 2 (8.3%) experienced tumor recurrence. Conclusion The RMS of the head and neck often presents with nonspecific symptoms. Individualized multimodal therapy should be performed for these patients, including surgery, chemotherapy and radiotherapy.