Saccular aneurysms of the aorta in childhood are rare, and the low incidence of aortic aneurysms among children limits our understanding of their aetiology and surgical indications. In this case report, we describe the successful surgical treatment of a 5-year-old boy with severe aortic valvular stenosis, supra-valvular aortic stenosis, and a large saccular aneurysm in the anterior wall of the ascending aorta, without any connective tissue disorder.