Pulmonary atresia with ventricular septal defect (PA + VSD) is a cyanotic congenital heart disease, also classified as Tetralogy of Fallot with pulmonary atresia. PA + VSD accounts for about 1–2% of congenital heart defects. The intracardiac anatomy is similar to tetralogy of Fallot but there is no direct communication between the right ventricle and pulmonary arteries. Major problems with surgical treatment are related to complexity of the pulmonary vascular bed. We report a case of a 50-year-old woman with congenital heart disease who was admitted to our Centre in July 2012. Congenital heart disease was first diagnosed at the age of 28 and at that time it was classified as a pulmonary valve atresia with ventricular septal defect with right-to-left shunt and common arterial trunk. Diagnostics performed in our Centre confirmed complicated anatomy of vessels in the chest, especially narrow and hypoplastic major aortopulmonary collateral arteries arising from descending aorta and left subclavian artery. The congenital heart disease was reclassified as a pulmonary atresia with ventricular septal defect.