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An 11-year-old girl was admitted to our hospital with a history of fatigue, effort dyspnea, and chest pain. On transthoracic echocardiography, the parasternal shortaxis and apical four-chamber color Doppler flow imaging showed a structure matching the level of the coronary sinus and pouring into the right ventricle. On multi-slice computed tomography, the circumflex artery became immediately narrowed just before draining in to the base of the right ventricle, close to the septal leaflet of the tricuspid leaflet, and the Qp/Qs was 1.6. Congenital coronary-cameral fistula is an infrequent condition that establishes a direct link between coronary arteries and cardiac chambers. Although coronary artery fistulae are commonly asymptomatic, they may cause severe symptoms depending on the severity of the shunt, and may be treated medically, surgically or by transcatheter closure. In this case report, we present a patient with a huge circumflex artery-to-right ventricle fistula treated successfully with a duct occluder device.