Rare congenital malformation can lead to the aberrant communication between systemic arterial circulation with pulmonary venous circulation which can range from sequestration to arteriovenous fistulas. We describe a 24 year old female with no significant medical history presenting with dyspnea and hemoptysis. On Computerized Tomography (CT) and angiogram found to have an aberrant vessel branching from celiac axis supplying the right lower lobe and draining into the inferior pulmonary vein with evidence of vascular steal. Due to the size of the vessel, decision was made to forego coil embolization and underwent Video-Assisted Thoracoscopic Surgery (VATS) ligation of the aberrant vessel. She had an uncomplicated operative course and post operatively had resolution of her symptoms. This report demonstrates a rare anatomical congenital vascular malformation causing hemoptysis and resolution of symptoms after ligation of the aberrant blood vessel. Keywords: pulmonary arteriovenous fistula; vats; hemoptysis.