Submit: Abnormal coronary artery birth from the pulmonary artery (PA) is a rare disease, characterized by age-dependent clinical forms. We will describe the clinical, electrical and echocardiography features and the management strategy of this disease in adults. Materials and methods: We enrolled 9 patients, 6 with the diagnosis of ALCAPA and 3 of ARCAPA. The mean age was 32 years with a sex ratio of 1.5/1. The presentations consisted of exertion symptoms and incidental findings. LVEF was reduced in 33% of patients with ALCAPA. Moderate mitral regurgitation (MR) was found in 44% of cases, particularly in cases of ALCAPA. Coronary CT scans confirmed that all our patients had coronary artery anomalies from the PA. The surgical treatment of coronary reimplantation was the therapeutic method of choice and was performed in 88% of our patients. Conclusion: Coronary birth defect from PA is a very rare congenital pathology. The clinical symptomatology that leads to the discovery of the disease is diverse. Doppler imaging and CT scann is a key tool in the positive diagnosis. Early re-implantation surgery can avoid complicated forms.