Persistent left superior vena cava is a malformation of cardinal veins. We report a case of a secundigravida who had many fetal ultrasound examinations – first performed by an obstetrician (who described fetal mediastinum as "abnormal”), second by other obstetrician, who performed basic fetal echocardiographic examination and diagnosed persistent left superior vena cava. The woman was referred to a tertiary center for detailed fetal echocardiography. The diagnosis of persistent left superior vena cava with agenesis of the right superior vena cava was confirmed. The anomaly had no influence on fetal hemodynamic stability, fetal life, delivery and early postnatal period. After delivery, the neonate was under observation for further anomalies, aortic coarctation in particular. Prenatal and postnatal management was summarized. Literature review is presented.