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Extrahepatic portal vein obstruction (EHPVO) refers to the cavernous transformation of the portal vein (also called “ portal cavernoma”) following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic. This case report demonstrates the ultrasonography appearances of cavernous transformation of the portal vein in a 3-year-old child.