Congenital subvalvular aortic stenosis may be associated with anomalies of the mitral valve. In this case, we present a patient with severe mitral valve regurgitation due to a perforation in the anterior mitral leaflet detected 4 months after an operation for relief of subaortic stenosis. A 10-year-old male patient who was operated for subvalvular aortic stenosis in another clinic was admitted to our hospital, and transthoracic echocardiography revealed severe mitral valve regurgitation due to a defect that was demonstrated at the anterior valve leaflet. The perforated area at the mitral valve zone A1 was repaired with a PTFE patch. The patient was successfully operated for the mitral valve perforation and the postoperative course was uneventful. In our case, the perforation in the anterior mitral leaflet implies a possible implementation of inappropriate surgical technique which necessitated a second surgical intervention after the initial operation.