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Fifty patients with advanced degrees of keratoconus, requiring corneal transplantation, were screened for mitral valve prolapse by two dimensional echocardiography. The overall prevalence of 58% was found to be statistically higher than the prevalence of 7% found in a group of age and sex-matched controls. It was also found to be higher than the previously reported prevalence of 38% in a group of keratoconus patients with similar age and sex match to our series. The findings of our study in conjunction with the histopathological and biochemical similarities between the two conditions strongly suggest that they may be different manifestations of similar defects in collagen metabolism.