Keratoconus is a noninflammatory disorder characterized by ectasia of the central or inferior portion of the cornea. This review presents the scant epidemiological information known to date and the factors believed to cause the development of the disease. They are the genetic factors for which evidence come from family studies, twin studies and genetic loci. There appears to be multiple genes causing a keratoconus phenotype with variable penetration. However, the genetic predisposition might not be enough; environmental factors, such as eye rubbing, atopy and UV exposure, may have a role in generating the disease.