We herein report the case of abnormal umbilical-venous return in which the antenatal ultrasound enabled us to establish the diagnosis of umbilical-systemic shunt (Type 1 according to Achiron (Achiron and Kivilevitch, 2016)). Due to the concomitant associations of cardiomegaly, intrauterine growth retardation, oligohydramnios, and left-lobe hypoplasia with agenesis of the intrahepatic umbilical vein – left portal vein – ductus venosus, a poor prognosis (11.1% survival) was to be expected. In spite of development of pulmonary arterial hypertension at birth, which was promptly treated, the evolution was nevertheless good, both on clinical and ultrasound follow-up.