Pulmonary arteriovenous malformation (PAVM) is a congenital vascular abnormality that can cause persistent cyanosis in children. PAVMs can go undetected till adulthood; however, there have been several neonatal cases reported over the years. This case report describes a classical manifestation of a child with isolated PAVM whose diagnosis was likely missed during the neonatal period. A high level of clinical awareness of this condition is crucial as early diagnosis and appropriate treatment can prevent lifethreatening complications and mortality. Diagnosis was confirmed by computed tomography pulmonary angiography and percutaneous transcatheter embolotherapy resulted in complete resolution of symptoms.

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