BACKGROUND Dissection of interventricular septum by aneurysm of the sinus of Valsalva is extremely rare. We present our experience with the management of 10 patients with this condition. METHODS Ten patients with aneurysm of the sinus of Valsalva dissecting into the interventricular septum were managed at All India Institute of Medical Sciences, New Delhi, between May 1987 and September 1996. Conduction abnormalities and aortic insufficiency dominated the clinical picture. Eight patients underwent surgical repair. Two patients refused operation, and only permanent pacemaker implantation was done for complete heart block in both these patients. RESULTS There was no hospital mortality. Follow-up ranged from 1 to 9 years. There was one late death due to carcinoma of the larynx, and 1 patient required reoperation for persistent aortic insufficiency. All other patients who underwent operation are in New York Heart Association functional class I. CONCLUSIONS We recommend surgical repair of this condition to deal with aortic regurgitation and to avoid the potential risk of rupture, thromboembolism, and infective endocarditis. However, surgical repair offers no guarantee against arrhythmias and conduction abnormalities.