Smooth muscle tumour (SMT) composed of leiomyoma and leiomyosarcoma recently has been described in many HIV-infected children. Leiomyosarcoma has become the second most frequent malignancy in children with HIV infection or other immunodeficiency diseases in the United States. Although leiomyosarcoma accounts for only 2% to 4% of childhood soft tissue sarcomas, the prognosis is poor in HIV-infected compared with non-infected patients. The development of Epstein-Barr virus (EBV)-associated SMT in children with acquired immunodeficiency virus (AIDS) decreases health, reduces quality of life, and often results in death. Some researchers, therefore, ascribe cause of death to SMT in the majority of these cases, not to AIDS. Currently, the optimal therapeutic strategy is controversial and there is a need to identify the efficacy and safety of different interventions for AIDS-associated SMT on overall survival and disease-free survival in children.