We present a rare case of spontaneous closure of fistula between right coronary artery and right ventricle in female infant. At the age of 7 days continuous murmur intensity 3/6 was registered in 3rd intercostal space along right sterna border. Echocardiography indicated, with high probability, a coronary artery fistula. Definitive diagnose was made at the age of 1 month with aortography. Radioscintiography and oxymetric analysis revealed 47% of left-to-right shunting at level of fistula. Because of the size of the shunting a surgical correction had been planned, but in mean-time the murmur had gone. At the age od 6 months control aortography showed spontaneous closure of fistula, but with significant morphological changes in the right coronary artery (stenosis and aneurisms). Exact mechanism of spontaneous closure is unknown, nor is the timing of therapeutic intervention. Control coronarography after 1 year showed regression of pathological changes in right coronary artery. Based on the literature and own experiences we consider invasive therapy indicated in children with significant clinical symptoms. In cases were clinical symptoms are absent expectative state should be taken, because there is chance of spontaneous closure of fistulas, even larger ones, as it is in our case.