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Polycystic kidney disease is an autosomal dominant genetic disorder, hence the name autosomal dominant polycystic kidney disease. It is the most common genetic kidney disease but is underestimated in our region. Cystic localisations can concern all organs and practitioners must think about it in any case.Liver involvement is the most frequent extra-renal manifestation. Estrogen-progesterone contraception and consanguineous marriages are some of the risk factors in families with a family history of PKHR.Its morbidity and mortality are mainly related to renal involvement.