A patient with generalized ochronosis developed severe cardiovascular symptoms related to ochronotic deposits on the aortic valve and in the coronary arteries. A transvalvular gradient of 100 mm Hg and obstruction of the left anterior descending coronary artery were found by catheterization. Aortic valvular replacement and aortocoronary bypass were performed successfully, and the patient has been well two years since operation. This represented the first reported case of aortic valve replacement for this rare metabolic condition. With increased mobility of these patients due to aggressive orthopedic joint treatment, more patients with this syndrome may require surgery for cardiovascular symptoms.