The persistence of superior left vena cava (PLSVC) is a pathological condition in fetus with risk of association with abnormalities like heterotaxy, cardiac abnormalities - atrioventricular septum defect, and conotruncal anomalies. In this paper we report 23 cases of fetuses with PLSVCs, reviewing their diagnosis, co-morbidities, and evolution in the newborns.