Cholestatic jaundice due to Ormond's disease (primary retroperitoneal fibrosis)

A case of a 51-year-old man, who presented with cholestatic jaundice due to common bile duct compression produced by primary retroperitoneal fibrosis, is reported. The patient was operated on two years earlier because of hydronephrosis, when the disease was diagnosed. Cholangiopancreatography revealed a "double duct sign", disclosing another benign cause for this radiographic sign, that once was considered to be pathognomonic of pancreas cancer. The bile duct stricture was managed by the insertion of an endoscopic endoprosthesis. This is the first report of jaundice due to Ormond's disease and the third, due to retroperitoneal fibrosis.