Scimitar syndrome is rare, but knowledge about it is important to pediatricians and pneumologists. X-ray can suggest diagnosis, but the interpretation can be wrong. If a diagnosis is made, further anomalies must be investigated with echocardiography and angiography, even if he patient is asymptomatic. This child showed evidence of pulmonary hypertension and required surgery. Postoperative evolution was excellent.

Keywords: Scimitar syndrome. Surgery. Pulmonary disease (specialty). Diagnosis.