Studies of families with inherited carcinomas have provided powerful tools to identify the genes involved in the pathogenesis of human cancers. In this review, we summarize the clinical, pathological, and genetic characteristics of the inherited carcinomas of the kidney. We emphasize the observation that different genes predispose to histologically different types of renal carcinoma. Hereditary papillary renal carcinoma, a recently described inherited disorder, is discussed in detail along with the predisposing gene, the MET protooncogene. The data support a classification of renal carcinomas based on molecular genetics.