A 9 year old boy presented an "idiopathic" retroperitoneal fibrosis. Fibrosis extended to the liver, compressing the major biliary tree, the portal vein, the urinary tract and the inferior vena cava. The process led two successive bilio-digestive shunts and was controlled after one year corticosteroid treatment. A malignant lymphoma was responsible for the death which occurred 4 years after the initial symptoms of retroperitoneal fibrosis.
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