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Cardiovascular abnormalities are infrequently documented in osteogenesis imperfecta, one of a group of hereditary, generalized connective tissue disorders. A patient with osteogenesis imperfecta is described with mitral valve prolapse, significant coronary artery disease and a coronary artery aneurysm. The latter two cardiac defects are apparently rare in this disease. The option of surgery was carefully considered with regard to technical feasibility and potential deterioration of the graft anastomoses. Although successful aortocoronary bypass surgery had not been previously reported in osteogenesis imperfecta, this patient received such surgery with therapeutic benefit. Therefore, coronary artery vascularization should be considered as a safe and effective treatment modality for patients with osteogenesis imperfecta and coexisting coronary atherosclerosis.