A 55-year-old male with hemophilia A came to the outpatient clinic with chest pain for several days after overdose injection of coagulation factor. He was a heavy smoker and a chronic alcoholic. An electrocardiogram (ECG) showed no specific change. A coronary computed tomography showed moderate stenosis with soft plaque at the distal segment of right coronary artery. His pain was improved with antianginal and reflux medications. Twenty days later, he ran to the emergency room complaining of squeezing chest pain. ECG showed mild ST segment elevation in inferior territories. Invasive coronary angiography via right radial artery revealed severe thrombotic occlusion at the same lesion. A bare metal stent was deployed and dual antiplatelet therapy including aspirin and clopidogrel had been maintained for 6 months under the conventional hemophilia management. The patient did not develop any coronary events just with single clopidogrel therapy for 5 years until he passed away from pancreatic cancer. Our case implicates that the invasive coronary intervention and post-procedural management could be safely performed with conventional standards of care while maintaining the usual dose of coagulation factors in a hemophilia patient with acute coronary syndrome.