This study investigated the incidence and development of pneumoconiosis in the Xinjiang Uygur Autonomous Region and cases reported by the Urumqi Railway Bureau to provide a scientific basis for developing prevention and control measures against pneumoconiosis. Data from pneumoconiosis cases were input into Excel and analyzed by SPSS version 17.0. There were 13,165 cases of pneumoconiosis through 2010. Coal workers accounted for the largest proportion of cases. From July 2006 through 2010, a total of 1233 new cases of pneumoconiosis were reported in the Xinjiang Uygur Autonomous Region; most cases were reported in Urumqi. From 1981 to 2012, 3332 new cases of pneumoconiosis had been confirmed by the Urumqi Railway Bureau, including 77.73, 16.96, and 5.31% stage I, II, and III cases, respectively. In the last 30 years, the number of new pneumoconiosis cases peaked in 1986; most of them were silicosis cases. In addition, there were more than 200 cases of pneumoconiosis combined with pulmonary tuberculosis reported by the Urumqi Railway Bureau. The coal industry in Urumqi is the main industry in which occupational pneumoconiosis occurs in Xinjiang. Thus, substantial effort is still required to eliminate pneumoconiosis by 2030.