The polycystic kidney disease is a hereditary malformation seldom described in Africa. Nineteen cases among 6 men and 13 women were seen in the internal Medicine Service in seven years. The range of age was 28 to 77 years. Ten other patients (of whom four were less than 15 years old were discovered via systematic family investigations and confirmed by ultra sound. The diagnosis was based essentially on consistent clinical signs and the presence of at least 5 cysts in each kidney. Infectious manifestations were the most frequent, presented in 37% of the cases. These acute infections can affect either the urinary ducts or a hepatic or renal cyst. The family investigations established that a third of the subjects examined were asymptomatic or had lumbar pain and hematuria never associated with their underlying cause.