12 cases including 11 males and 1 female of rhabdomyosarcoma of ocular adenexa were analysed. The average age of the patients was 4.2 years. 9 cases of lesion were located in the orbit, 2 in the eyelid and 1 in conjunctiva. Pathologically 8 cases were embryonal forms, 2 alveolar, 2 polymorphic. Immunohistochemical studies were performed in 8 cases, which showed desmin positive. Myoglobin presented strong or weak positive in 5 cases, suspicious positive in 3 cases. The clinical manifestation, prognosis and treatment were briefly discussed.