This study was aimed at determining the median survival and most frequent causes of death in patients with the autosomal dominant polycystic kidney disease (ADPKD). A retrospective, observational analysis was made on patients registered with a diagnosis of ADPKD, in the computer records of the Sheffield Kidney Institute (SKI), United Kingdom, during the years 1981 to 1999. Data on 363 patients were analyzed from these computer records and further information, if any, was obtained from the patients' clinical notes. During this period, 88 patients died. The median age of the patients who died was 60.5 years, with the youngest being 37 years old and the oldest being 82 years. The major causes of death in this study group were cardiovascular (46.6%), infection (15.9%), central nervous system (CNS) disorders (11.36%), and miscellaneous causes (11.36%). Our study suggests that the major cause of death in patients with ADPKD was cardiovascular followed by infection, of which 42% of the deaths were due to septicemia. CNS causes of death comprised 11.36% of whom 60% had cerebrovascular events including subarachnoid hemorrhage in 20% of the patients. Uremia was the cause of death in only 2.2% of the patients in this series.