Anomaly of the left anterior descending (LAD) coronary artery arising from the right sinus of valsalva is frequently seen with tetralogy of Fallot (TOF). The association of the LAD coronary artery with ventricular septal defect (VSD) is uncommon. We described an anomalous origin of the LAD coronary artery from the right sinus of valsalva with ventricular septal defect in a 38-year-old male patient suffering from atypical angina. The LAD coronary artery arose from the right sinus of valsalva, just next to the right coronary artery. There was a single opening in the membranous part of the interventricular septum. From this case, we suggest that angiography is useful for both documenting anomalies of the LAD coronary artery associated with VSD and for determining the safest surgical procedures.