A 63-year-old man was admitted with complaints of exertional dyspnea and atypical chest pain. Coronary angiography and 64-slice multidetector computed tomography (MDCT) revealed an anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA). He received a coronary artery bypass graft (CABG). The incidence of ARCAPA is extremely rare. We report here on the first case of ARCAPA that was noninvasively diagnosed and postoperatively followed up with 64-slice MDCT.