Congenital coronary arteriovenous fistula is uncommon disease, and was first described by Krause in 1865. About 20% of the cases, it associates additional congenital heart diseases. A 5-year-old female patient was diagnosed as coronary AV fistula with VSD, and was taken surgical correction under cardiopulmonary bypass. VSD was small and subarterial in type, and the fistula was dilated as adult thumb tip size at its distal portion. VSD was closed directly through the pulmonary arteriotomy and the aneurysmal dilation was opened vertically, then it was obliterated using 5-0, 6-0 prolene continuous suture fashioning into a long slender tube. Postoperative course was uneventful.