Autosomial dominant polycystic kidney disease (ADPKD) is an hereditary affection transmitted in a autosal dominant pattern with variable penetrancy. Diagnosis is based upon ultrasound examination and/or familial history. The authors present a 8 years retrospective hospital study in order to precise the epidemiological and clinico-biological aspects of ADPKD in Dakar. So 23 patients were studied. The mean age at the diagnosis was 46 years. They were 9 men and 14 women (sex ratio 0.64). A past history of familial nephropathy was found in 52.2% of cases. The circumstances of diagnosis were dominated by lombar pain (52.2%) and hypertension (17.4%) but at examination, blood pressure was high in 34.8% and represent the main factor of worsening the disease. Enlargement of kidney was found in 47.8% and hepatomegaly in 13% of cases. Anemia was particularly common 56.5%. The most frequent extra renal manifestation was the liver cysts and occurs mainly in women. National prevalence of ADPKD and the specific gene should be precise in further studies.