The Rastelli operation has been the most common procedure for the repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. A relatively recent approach is the Nikaidoh procedure. Despite the fact that it seems promising, the operation lacks long-term follow-up data. It has been postulated that patients with anomalous coronary arteries are high-risk candidates for the Nikaidoh procedure and its modifications. In this report, we present the case of a patient with transposition of the great arteries with remote restrictive ventricular septal defect and left ventricular outflow tract obstruction with coronary anomaly - with the right coronary artery originating from the left anterior descending coronary artery and crossing the right ventricular outflow tract - who underwent successful modified Nikaidoh operation.