Absent pulmonary valve (APV) is a relatively rare congenital heart disease, and is mostly associated with tetralogy of Fallot phenotype or ventricular septal defect. APV with intact interventricular septum (IVS) is even less common with case reports or very small series in the literature. Congenital aortic regurgitation with a rudimentary non-coronary cusp is also by itself a rare congenital anomaly and to our knowledge this is the first report of the combination of APV, intact IVS, abnormal aortic valve and ascending aortic aneurysm. The clinical course, possible etiologies and management are discussed.