The authors report a case of congenital extra-hepatic portocaval shunt in a two-year-old girl. While pointed out antenatally by ultrasound and suspected by a follow-up ultrasound at the age of two years, this anomaly could be confirmed only by coupled ultrasound scanning and color Doppler, which allows for dynamic anatomic and morphologic evaluation. Comments concern mainly the embryologic hypothesis and the clinical tolerance of this malformation (because no therapeutic measures were adopted).