<p>Anomalous origin of coronary arteries from the pulmonary artery (ACAPA) are rare but clinically significant condition with high mortality if left untreated. Even more rarely, ACAPA is associated with other congenital heart defects. From 1974 to 2024, 120 patients with anomalous coronary arteries connected to the pulmonary artery were retrospectively analyzed. Medical records including surgical operative notes and angiography protocols were screened for any other cardiac and extracardiac defects. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) was present in 103 patients, anomalous right coronary artery connected to the pulmonary artery (ARCAPA) in 6, anomalous circumflex coronary artery connected to the pulmonary artery (ACXPA) in 7, anomalous left anterior descending coronary artery connected to the pulmonary artery (ALADPA) in 2, and anomalous single coronary artery connected to the pulmonary artery (ASCAPA) in 2 patients. Anomalous origin of the coronary artery from the pulmonary arteries was associated with other congenital heart defects in 16 patients (13%) and with extracardiac anomalies in 10 patients (8%). Most associated cardiac anomalies were left-sided obstructive defects or shunt-lesions. Patients with ACAPA and associated cardiac defects had poorer perioperative survival. A precise diagnosis of coronary anatomy is crucial for preoperative planning and the success of the surgery of patients with congenital heart defects. In particular, for patients with a challenging postoperative course, an anomalous coronary artery originating from the pulmonary artery should be considered.</p>