Keratoconus is an ectatic disorder with highly complex and varied causes including genetic variations and environmental factors. Its prevalence varies widely between regions and countries. Many environmental factors have been proposed to be associated with keratoconus, but the interpretation of their individual contributions is difficult due to the presence of many confounding variables. The current literature was reviewed to evaluate the strength of the associations and the causative effects of environmental factors on keratoconus. Ethnicity and consanguinity have been revealed as important determinants for geographical variations in keratoconus prevalence. Eye rubbing, atopy, floppy eyelid syndrome, contact lens wear, pregnancy, and thyroid hormone disturbances are likely associated with keratoconus. The first 4 factors can induce ocular surface inflammation, matrix metalloproteinase release, and keratocyte apoptosis, consistent with the postulated etiology of keratoconus. The associations of keratoconus with UV exposure, cigarette smoking, personality, and sex were less convincing once confounding factors were considered. Future studies powered for multivariate analysis of factors discussed will hopefully shed light on what is truly important in the development and progression of keratoconus.