We report a new-born case of total conus defect type ventricular septal defect (VSD) and single coronary artery with situs inversus totalis, suspected Kartagener syndrome clinically. After the birth, as the patient had suffered from respiratory distress due to high pulmonary blood flow through the large defect, surgery was planned at age of 14-days after birth. Under median sternotomy and cardiac arrest, patch closure of VSD was performed as ordinary fashion. In spite of the situs inversus totalis and single coronary artery arose from right coronary sinus, operator could have completed all of surgical procedure at the right side of patient as usual. No remarkable respiratory complication was seen postoperatively and she was discharged from hospital 18th day in a good condition.