We report two cases of ochronosis. This rare disease (1/1,000,000) transmitted by recessive autosomic inheritance results from an enzyme disorder. Clinically, the disease begins by black deposits in connective tissue followed by a group of symptoms, particularly involving the joints, and then destructive joint disease affecting the larger joints. Diagnosis is often established late. Early detection is important, preoperatively if possible, in order to avoid the serious complication of infectious endocarditis. These patients require adapted multidisciplinary care associating social support and symptomatic treatment. Drug therapy is currently under study and appears to provide effective symptom relief.