In this study, a genetic model of polycystic kidney disease (PKD) has been produced in transgenic mice bearing the murine c-myc gene driven by the SV40 enhancer and the adult beta-globin promoter. These animals reproducibly develop PKD and die of renal failure. The phenotype appears to result from the overexpression of c-myc in the renal tubular epithelium and consequent abnormal cell proliferation. These transgenic mice represent a genetic model of PKD which bears similarities to human autosomal dominant PKD (ADPKD) with respect to renal morphology, renal functional alterations and dominant transmission. Study of these transgenic mice may offer valuable insights into the pathogenesis of PKD.