Cavernous transformation of the portal vein (CTPV), known as portal cavernoma, is caused by stenosis or obstruction of the portal vein. This condition leads to spontaneous bypass across the stenosis, which sustains blood flow and liver function. Most cases of CTPV in children are caused by neonatal septicemia, and umbilical and intra-abdominal infections. However, CTPV has been rarely reported after choledochal cyst surgery. The traditional treatment for CTPV is surgery. We report a case of successful treatment of a CTPV by percutaneous transhepatic angioplasty.