Objective To discuss the methods and results of the surgical treatment of apical muscular ventricular septal defects （VSD） , in order to improve the surgical outcome of this disease. Methods The clinical data of 29 patients with apical muscular VSD of Fuwai hospital through Jan. 1999 to July 2004 were analyzed retrospectively. The apical VSDwere repaired via 4 different approaches： right atrium, apical right ventriculotomy, apical left ventriculotomy and the incision of the outflow tract of right ventricle combined with apical left ventriculotomy. The operative outcomes between different approaches were analyzed and compared. Results There were 2 perioperative deaths （6. 9%）. One patient repaired via apical left ventriculotomy died of low cardiac output syndrome, the other died intraoperatively from endocardial fibroelastosis, which was confirmed by myocardial biopsy. There were residual VSD in 4 （13. 8%） patients, who were repaired via the right atrium, which did not necessitate reoperation. Other patients were discharged uneventfully. Follow up was completed {n 11 patients and extended 2 to 34 months, none of the 6 survivors had apical ventricular aneurysms. Conclusions The operative outcome of patients with apical VSD is satisfactory although the incidence of residual VSD is higher. The intraoperative transesophageal echocardiography （TEE） should be performed routinely. Surgical approach should be chosen on the actual site of the VSD at operation. Try to repair the VSD via the right atrium as possible, via the apical right ventrieulotomy if needed and try to avoid left ventriculotomy.