Congenital coronary sinus (CS) stenosis is a rare malformation. We present five patients with congenital coronary sinus stenosis (CSS) and identified another nine cases reported in the literature between 1980 and 2016. Congenital CSS may be associated with an unroofed CS, a coronary artery–coronary sinus fistula, or other cardiac anomalies. Congenital CSS may be detected by echocardiography, multidetector row computed tomography, and intra‐operative evaluation. Congenital CSS can occur at the ostium, lumen, and origin of the CS. Congenital CSS imaging features, treatment, and outcomes are summarized herein.