The authors report a retrospective series of 217 cases of autosomal dominant renal polycystic disease collected over a period of 30 years in the urology and nephrology departments of Nantes university hospital. They study the incidence of urological complications, observed in 87 patients (40%), consisting of calculi (15%), infection (22%, with 4 deaths), intracystic haemorrhages (3.5%) and urinary tract compression (2%). The diagnostic and therapeutic methods are presented and discussed. The results of renal transplantation are also analysed: 39 patients were transplanted, 72% retained a functioning kidney with a mean follow-up of 44.9 months (range: 12-108 months) and three patients died as a result of infectious complications. The 1-year and 3-year actuarial transplant survival rate of 92% was similar to that of renal transplantations performed for another form of renal disease. Preparation for renal transplantation remains an essential problem: the two major indications for pre-transplantation nephrectomy were the size of the kidneys and the presence of infection.