Coronary artery to pulmonary artery fistula (CA-PAF) is a rare congenital anomaly. The purpose of this retrospective study was to analyze 11 adult patients with CA-PAFs treated surgically, and to evaluate the surgical management and long-term results. There were no surgical deaths and all patients survived the follow-up periods (mean 7.2 years). All symptomatic patients improved their New York Heart Association functional class. As surgical correction is safe and effective, with good long-term results, all the patients with CA-PAF in adults can be candidates for surgery to prevent life-threatening complications.