BACKGROUND The authors herein report surgical experience with the aneurysms of sinus of Valsalva (SVA) complicated by coexisting pathologies. METHODS Eight patients aged between 11 and 55 years underwent surgical repair of SVA. The aneurysms originated from the right coronary sinus in four patients, from the non-coronary sinus in four patients and from the left in one patient (one patient had aneurysms originating from both the left and right sinuses). Six of the aneurysms were ruptured into the right atrium (n = 3), right ventricle (n = 2) and left ventricle (n = 2). Aortic insufficiency (AI; n = 3), ventricular septal defect (n = 2), atrial septal defect (n = 4), Marfan's syndrome (n = 2), pulmonary stenosis (n = 1) and aortic stenosis (n = 1) were the coexisting anomalies. Double-chamber exposure (right atrial/ventricle and aortic) was used in all of the patients. Patch closure was preferred for defect closure. In three patients with AI, valve replacement was necessary. Two patients with endocarditis in New York Heart Association classes III-IV underwent urgent operation. RESULTS The patient with annular narrowing was the only in-hospital death due to severe infection. The survivors were followed up for 85 months (range: 6-156 months). In two patients with Marfan's syndrome reoperation was necessary. All the surviving patients were asymptomatic with no unfavourable consequences. CONCLUSIONS Although SVA can be treated successfully with low operative risk, the factors that influence patient outcome include infective endocarditis, Marfan's syndrome and the preoperative functional status of the patient.