Recurrent pyogenic cholangitis (RPC) is characterized by repeated attacks of bacterial infection of the biliary tract by enteric organisms resulting in formation of strictures and stones in the intrahepatic as well as the extrahepatic bile ducts. Between 1973 and 1984, we managed ten children with RPC. Presentation was acute, septicemia being invariably present. Four children responded to vigorous intravenous (IV) fluid and antibiotic therapy; of these, three subsequently required elective transduodenal sphincteroplasty. Six children required emergency biliary tract decompression surgically and three of them also had concomitant definitive drainage procedures (transduodenal sphincteroplasty, two; supraduodenal choledochoduodenostomy, one). The other three had T-tube drainage initially followed by elective drainage procedures (transduodenal sphincteroplasty, one; supraduodenal choledochoduodenostomy, two). At exploration, pigment stones and/or mud were invariably present in the bile ducts. One child required reexploration for hemostasis following sphincteroplasty, and one child died from septicemia and respiratory failure following operation. The rest are well, having been followed for 3 to 12 years. Major advances in recent years include (1) endoscopic retrograde cholangiopancreatography (ERCP) for accurate definition of the biliary tract and confirmation of the presence and location of stones during the quiescent phase, (2) the intraoperative choledochoscopic extraction of intrahepatic and extrahepatic stones, and (3) postoperative stone removal via the T-tube tract.