Primary cilia (PC) are antenna-like organelles that protrude from most mammalian cells. They are essential for the regulation of several signaling pathways such as Hedgehog and WNT It is therefore not surprising that a dysfunction of PC is frequently associated with pathologies. Originally, PC were found to be involved in a variety of diseases commonly referred to as ciliopathies including cystic kidney diseases. Evidence is accumulating that PC play also an important role in cancer formation and regulation, which is the focus of this review.