ABSTRACT
Unilateral pulmonary artery agenesis is a rare malformation. It can lead to several complications.The diagnosis is usually set at adolescence, however it can remain asymptomatic and late diagnosis is possible. Diagnosis is based on thoracic angioscanner. Treatment is essentially symptomatic. A 20-year-old male patient presented to our clinic with a history of cough and dyspnea with an abnormal chest X-ray. Physical examination was normal. Chest X-ray, CT-Scan and Pulmonary ventilation-perfusion scintigraphy led to the diagnosis of right pulmonary artery agenesis. Unilateral agenesis of the pulmonary artery with pulmonary hypoplasia is a rare malformation whose prognosis can be fatal. Once the diagnosis has been established medical follow up is mandatory.