Anomalous origin of left coronary artery (LCA) from the right coronary cusp with an intramural course is usually managed with unroofing of the intramural segment. Available literature demonstrates an uneventful course following surgery in most patients. Coronary stenosis following the unroofing procedure treated with percutaneous coronary intervention has not been described in the past. We describe a case where an 11-year-old girl with anomalous origin of the LCA from the right coronary cusp presented with near syncope. Surgical unroofing of the intramural segment was done without any post-operative complications and the patient remained asymptomatic for 9 months. She then presented with chest pain, abnormal troponin levels, and ST-T wave changes on EKG. A CT angiogram done revealed short segment narrowing of the LCA near its origin. Cardiac catheterization with coronary angiography demonstrated short segment narrowing of the LCA just distal to origin. Stenting of the left main coronary artery was done with a drug eluting stent. She underwent the procedure without complications. The patient continued to be asymptomatic 16 months after placement of the stent and there was no residual stenosis seen on a repeat CT angiogram at 3 months after the procedure.