GE There are two forms of polycystic liver disease: polycystic liver disease in isolation, in which patients have cysts only in the liver, and autosomal dominant polycystic kidney disease, in which patients have cysts in both the liver and the kidney. The latter form is more common, representing 80–90% of all polycystic liver cases. Both forms of the disease are genetically determined. In patie...

An autosomal dominant pattern of transmission has been established for polycystic kidney disease. The degree of cystic involvement of other organs has been variable. The genetic pattern of transmission of polycystic liver disease independent of cystic kidney disease has never been established. We present a second family with polycystic liver disease without kidney disease. The lack of renal cys...