Mild form of osteogenesis imperfecta (OI) may have a normal life span. However, cardiovascular complications including aortic and valvular heart disease, and coronary artery disease may complicate the life period. We presented a patient with mild form of OI and premature coronary atherosclerosis. He had been performed primary percutaneous angioplasty and drug eluting stent implantation to left anterior descending coronary artery osteal lesion. Then he presented with unstable angina pectoris due to the diffuse in-stent restenosis and a highly critical lesion adjacent to previously stented segment. He was suggested coronary artery bypass grafting (CABG), but he preferred coronary angioplasty and was implanted everolimus eluting stent. Control angiography, performed at 9th month, revealed the everolimus eluting stent was satisfactorily patent and the patient was asymptomatic. Numerous genetic defects and histopathological abnormalities of collagen and bone formation that were reported in the etiology of OI may be accounted for premature atherosclerosis in OI. Patients with mild form of OI may present with premature atherosclerosis and acute myocardial infarction. Everolimus eluting stent implantation may be a better choice of drug eluting stent in patients with OI instead of other drug eluting stent or minimally invasive CABG.