A rare cause of aortic valve stenosis: Ochronosis.

Ochronosis is an autosomal recessive metabolic disorder which may cause aortic valve stenosis. In this article, we report a 58-yearold male patient with alkaptonuria who presented to our institute with severe aortic valve stenosis. He underwent successful aortic valve replacement with a mechanical prosthesis. The deposition of foreign material in valvular tissue is a rare case scenario which may result in serious problems, the management of which may necessitate valve replacement with prosthetic heart valves.