The importance of tubular epithelial hyperplasia in polycystic kidney diseases has become apparent during the last decade. Micropapillary hyperplasia occurs in autosomal dominant polycystic kidney disease, in localized cystic disease, and in acquired cystic disease. Neoplastic or severely dysplastic epithelial hyperplasia occurs in von Hippel-Lindau disease. A histopathologically distinctive epithelial hyperplasia occurs in tuberous sclerosis. In each of these conditions, epithelial hyperplasia may be related to cyst formation and may also impose an increased risk of malignancy--a risk that seems to be highest in patients under treatment with long-term hemodialysis for end-stage kidney disease. Although hyperplasia in some of these diseases may share a common pathway of development, it is more probable that the histopathologic differences reflect different pathogenetic pathways that converge on a common endpoint.