Autosomal recessive and autosomal dominant polycystic kidney disease (ADPKD and ARPKD) are 2 major variants of inherited diseases leading to the cystic degeneration of the kidney and other organs. Primary cilia have moved into the focus of research as it is becoming increasingly clear that dysfunction of this special cell organelle represents the main underlying mechanism of disease. At the same time, we also learn that the primary cilium plays an important role in the regulation of cell proliferation and transport processes along the nephron in the healthy kidney. Three recent publications implicate 2 novel players in ciliogenesis and cyst formation: the von Hippel Lindau tumor suppressor gene (pVHL) and collectrin.