Bronchopulmonary sequestration is an uncommon congenital anomaly. Although systemic arterial supply to the abnormal lung tissue is usually derived from the aorta, other nutrient vascular sources have also been described, including, infrequently, the coronary circulation. A right-sided aortic arch is another rare vascular abnormality, generally present in the setting of a vascular ring. We report a case of pulmonary sequestration receiving arterial supply from the right coronary artery, in the presence of a right-sided aortic arch, a constellation of anomalies not previously described.