The authors report two cases of polycystic liver andkidney in two women. They were unusual in that the liver first gave risr to symptoms and the renal lesions were completely latent. Both hepatic and renal lesions were well tolerated in spite of hepatomegally. They emphasize the interest of laparoscopy and biopsy of the liver under direct vision, and selective aortography, in the diagnosis. They discuss the basic differences between polycystic liver and kidney in adults, from that in children ornewborn, and caroli's disease. Finally, they emphasize the usually poor prognosis dominated by progressive renal failure which should guide treatment. The latter depends on periodic supervision of the patient for the disease usually evolves slowly. Treatment should therefore be conservative. Tn some cases, however, renal transplantaton should be considered before irreversible renal failure in young subjects, at an age when it is still possible. On the other hand, the liver lesions do not require any radical treatment, unless there is intercurrent infection or pressure on neighbouringorgans.