ABSTRACT   BACKGROUND፡ Unilateral pulmonary artery absence or discontinuity is a rare congenital abnormality. Its reported prevalence is 1 in 150,000 adults. CASE PRESENTATION: A 22-month-toddler girl was referred from a general hospital for cardiac follow-up to our hospital after diagnosis of severe pulmonary hypertension was made. She presented with generalized body swelling, cough, fast breathing and high-grade fever of four days duration. She had repeated severe chest infections. Echocardiography and chest computed tomography revealed right pulmonary artery discontinuity. Acute care was given and chronic care was established.  CONCLUSION:Pulmonary artery discontinuity is a rare anomaly. It should be considered in any child presenting with recurrent chest infections/pulmonary hypertension. Appropriate and timely diagnostic work up will avoid misdiagnosis. KEYWORDS: pulmonary artery discontinuity, right pulmonary artery atresia, Ethiopia