We report the case of a 41-year-old patient presenting with anomalous of origin of the right coronary artery from the pulmonary artery (ARCAPA) and severe aortic valve stenosis. Surgical correction was performed by partial (T-shaped) sternotomy. After replacing the bicuspid aortic valve, the right coronary artery ostium was directly reimplanted into the aortic root. The main pulmonary artery was reconstructed using an autologous pericardial patch. Postoperative recovery was uneventful. Four cases involving an association of the bicuspid aortic valve and ARCAPA have been reported in the literature. This potential relationship should be considered whenever diagnosing a bicuspid aortic valve.