Ochronosis is a rare disease. Usually symptoms appear in the third, fourth, or later decade of life. The most common symptom is arthropathy, but cardiovascular system can be involved in this disease. A 71-year-old man with ochronosis was scheduled for total hip arthroplasty. Preoperatively, echocardiogram revealed aortic valve stenosis, mitral valve stenosis, and hypokinesis on antero-septal, lateral and posterior walls. Coronary angiogram revealed 50-75% stenosis of the left anterior descending coronary artery (segment 7) and 100% stenosis of the left circumflex artery (segment 15). Before the induction of general anesthesia, electrocardiogram showed first-degree atrioventricular block. After the induction of general anesthesia, blood pressure decreased markedly. Phenylephrine administration and rapid infusion of extracellular fluid failed to increase blood pressure. Thus, we started to administer dopamine at an infusion rate of 10 microg x kg(-1) x min(-1) which increased blood pressure effectively, but electrocardiogram showed second-degree atrioventricular block (Mobitz type II). We started rapid infusion of a plasma substitute, and gradually decreased the infusion rate of dopamine to 4 microg x kg(-1) x min(-1). Then electrocardiogram returned to first-degree atrioventricular block. We estimated that second-degree atrioventricular block in this patient might have been exaggerated by dopamine at least in part.