INTRODUCTION: An anomalous origin and course of the right coronary artery (RCA)(1) is a very rare congenital anomaly that can be fatal if it remains undiscovered.

PRESENTATION OF CASE: In this case report, we present a patient with a one-year history of exercise-induced angina and dyspnea caused by anomalous origin of the RCA from the left sinus, and anomalous course between the aorta and the pulmonary artery.

DISCUSSION: Possible mechanisms of this disease's symptomatology are compression of the RCA between the aorta and the pulmonary artery in its anomalous inter-arterial course, and squeezing of the RCA in the proximal intramural part.

CONCLUSION: In this report, we present some unique images of the RCAs course, which contribute to the understanding of this disease's symptomatology. The patient successfully underwent surgery with Right Internal Mammary Artery to RCA (RIMA-RCA)(2) bypass with complete remission of all symptoms.