BackgroundBoth hypoplastic left heart syndrome and ventriculo‐coronary communication are extremely rare congenital cardiac conditions.CaseThe diagnosis of hypoplastic left heart syndrome combined with endocardial fibroelastosis and ventriculo‐coronary communication was made in a pregnant woman at 24 weeks of gestation. Interestingly, unlike diastolic flow from the coronary artery to the left ventricle in typical ventriculo‐coronary communication, this fetus showed a systolic flow from the left ventricle to the coronary artery.ConclusionTo the best of our knowledge, this is the first study to present only a systolic flow signal in hypoplastic left heart syndrome combined with ventriculo‐coronary communication in prenatal ultrasonography.