To appraise the frequency, clinical features and prognosis of adult polycystic kidney disease (APKD) in Hong Kong, 41 Chinese patients who had an autopsy diagnosis of APKD were studied retrospectively. The APKD patients constituted 0.295% of 13,890 (or one in 339) autopsies performed during the study period (1964-1991). The frequency of APKD in autopsied populations in Western countries varies from one in 261 to one in 1,019 with an average of one in 503. Nineteen (46%) patients reached end-stage renal disease (ESRD) at the time of death. Ruptured berry aneurysm caused death in 3 patients while 3 others had spontaneous intracerebral hemorrhage. Left ventricular hypertrophy was found in 24 (59%) patients. Polycystic liver was present in 26 (63%) patients. This frequency was higher than the 4% to 60% (average 47%) reported in similar studies conducted in Western countries. The probability of patients being alive and without ESRD, estimated using a life table analysis, was 83%, 54%, 32% and 12% by the age of 40, 50, 60 and 70 respectively. No difference in frequency, clinical features and survival between male and female APKD patients could be demonstrated.