Rhabdomyosarcoma of the maxillary sinus is uncommon in adults. The clinical course and appropriate treatment strategy for the disease remains to be elucidated. This article describes two adult patients with rhabdomyosarcoma of the maxillary sinus who achieved long-term survival after undergoing multimodal therapy. We also reviewed the literature regarding 23 patients who were 15 years of age or older and had rhabdomyosarcoma of the maxillary sinus and were treated between 1950 and 2000. Results of our analysis suggest that multimodal therapy may become the mainstay treatment for adult rhabdomyosarcoma of the maxillary sinus. We believe that adequate surgical procedures, planned radiotherapy, aggressive chemotherapeutic agents and the best supportive care for complications may improve the prognosis of patients with this disease.