The present study aimed to investigate the clinicopathological features of cases of classic Kaposi's sarcoma (CKS) in Xinjiang Uygur Autonomous Region, China, and analyze its etiology and treatment. A total of 114 patients, who were clinicopathologically diagnosed with CKS at the First Affiliated Hospital of Xinjiang Medical University (Urumqi, China) between 1980 and 2015 were retrospectively analyzed. The clinicopathological features of CKS were summarized, and its demographic distribution, pathogenesis, etiology and treatment were examined. The results revealed that, among the 114 patients with CKS, 100 patients were men and 14 patients were women, with a respective ratio of 7:1. The average age of these patients was 57.5 years old, and 97 of the patients were from the Uygur Autonomous Region (85.1%). Among the 114 patients, 60 patients (52.6%) were from Southern Xinjiang, 50 patients (43.9%) were from Northern Xinjiang and four patients (3.5%) were from Eastern Xinjiang. It was found that CKS in the Uygur ethnic group of Xinjiang Uygur Autonomous Region had unique clinicopathological features. The occurrence of CKS in Xinjiang may be associated with human herpes virus 8 infection, ethnicity‑based susceptibility and lifestyle.