This 1986-2006 study sought to determine whether specific causes led to increased mortality risks for Taiwanese workers with an approved compensation claim for permanent occupational disability (ACCPOD) of the upper or lower extremities. All cases of ACCPOD between 1986 and 2006 were collected from the database of compensation claims at the Bureau of Labor Insurance. Standardized mortality ratios and 95% confidence intervals were calculated for different causes of death among workers with an ACCPOD of the upper or lower extremities. A total of 800,047 person-years were accrued for 71,001 workers with a single type of disability. Standardized mortality ratios were significantly increased for all causes, including liver cirrhosis, injuries, and intentional self-harm. Standardized mortality ratios for workers with amputations of the lower extremities increased to 7.66 (95% confidence interval (CI): 5.36, 10.61), 2.40 (95% CI: 1.44, 3.75), 2.07 (95% CI: 1.03, 3.70), and 5.09 (95% CI: 2.20, 10.03) for those with diabetes mellitus, cerebrovascular disease, liver cirrhosis, and chronic renal failure, respectively. The authors concluded that workers with occupational disabilities involving an upper or lower extremity should be assisted to prevent further injuries or intentional self-harm, whereas those with lower limb amputations should be provided care related to proactive control of diabetes mellitus and associated complications during rehabilitation.