BACKGROUND: Autosomal dominant polycystic kidney disease is the most frequent inheritable kidney disease. Epidemiological studies have not been performed in Norway. MATERIAL AND METHODS: Based on the Norwegian Renal Registry, we retrospectively studied 375 patients diagnosed with this disease taken into renal replacement therapy between 1980 and 1997. They were compared with patients with glomerulonephritis. RESULTS: The average age for autosomal dominant polycystic kidney disease patients at start of renal replacement therapy was 55.2 years with a significant difference (p < 0.003) between men (53.7 years) and women (57.1 years). 295 patients (78.7%) were transplanted while 80 (21.3%) were not transplanted. From start of renal replacement therapy autosomal dominant polycystic kidney disease patients had a significantly higher (p = 0.0002) five year survival rate (70%) compared with glomerulonephritis patients (60%). One and five year patient survival rates were significantly higher (p = 0.0006) for living donor recipients (95% and 90%) compared with necro-kidney recipients (90% and 75%). One year graft survival rates for kidneys from living donors was 90% and 80% for necro-kidneys, after five years the graft survival rate was 80% and 65% (p = 0.0026). After an average of five years, 227 (60.5%) patients are alive, 197 with functioning kidney grafts (87%) while 30 (13%) are on dialysis. INTERPRETATION: Male autosomal dominant polycystic kidney disease patients start renal replacement therapy earlier than female patients. Autosomal dominant polycystic kidney disease patients do have better patient survival in renal replacement therapy than patients with glomerulonephritis.