Objective To analyze the clinical features of AIDS-related Kaposi's sarcoma (AIDSKS) patients in Xinjiang uygur autonomous region,and to evaluate the efficacy of highly active antiretroviral therapy (HAART) and systemic chemotherapy.Methods Clinical data on 70 AIDS-KS patients admitted in Sixth People' s Hospital of Xinjiang Uygur Autonomous Region from January 2008 to June 2013 were reviewed.The demographic characteristics,progress of KS,extent of lesions,treatment and prognosis of these patients were retrospectively studied.The efficacy of different therapies and the prognosis were compared using t or x2 test.Results The average age of 70 AIDS-KS patients was (40 ± 10) years,and the ratio of male and female was 2.5 ∶ 1.Sixty-six out of 70 (94.29％) patients were Uyghur ethnic.Among 70 patients,23 cases (32.9％) were rapidly progressed.Lesions often progress to plaques and nodules affecting head,neck,trunk,limbs and internal organs.Among 64 evaluable cases,the fatality rate in treatment group was 7.7 (4/52),which was significantly lower than that in patients who did not receive treatment (4/12,x2 =5.861,P ＜ 0.05).After 6-month of HAART or HAART sequenced by chemotherapy,CD4+ T lymphocyte counts were significantly increased (t =35.258 and 42.528,P ＜0.01),while it was decreased or remained in low level in patients received other treatments or untreated.HAART sequenced by chemotherapy,chemotherapy sequenced by HAART,and chemotherapy alone were effective for all AIDS-KS patients,while the effective rate in patients received HAART alone was 86.4％ (19/22),and immune reconstitution inflammatory syndrome (IRIS) was appeared in 45.5％ (10/22)patients.Conclusions AIDS-KS more frequently occurs in young Uighur males.The lesion may affect various organs,and some progress rapidly.HAART plus chemotherapy may reduce the incidence of IRIS and improve the prognosis.