Alveolar rhabdomyosarcoma (ARMS) commonly occurring in children and adolescents, is a rare and aggressive soft tissue malignancy. Here, a rare case of pleural ARMS is reported in a boy presenting dyspnea for two weeks. The pathology of a biopsy specimen from pleura showed ARMS. PAX3-FKHR fusion protein is positive and the PAX7-FKHR fusion protein is negative as detected by fluorescence in situ hybridization. The prognosis of this patient was poor and nonresponsive to chemotherapy.