OBJECTIVE This report describes our experiences with 5 cases of biliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia. METHODS A review of medical records was undertaken in an effort to recognize cases of biliary atresia associated with meconium peritonitis. RESULTS Five patients of 171 with biliary atresia (2.9%) were detected to have meconium peritonitis caused by perforation of small bowel atresia. The biliary atresia was not suspected during the initial operation for meconium peritonitis. Total parenteral nutrition (TPN) made it difficult to make an early differential diagnosis of biliary atresia because of the presence of TPN-associated cholestatic jaundice, and the Roux-en-Y limb used for hepatic portoenterostomy could not be made long enough to prevent cholangitis caused by preexisting short bowel. The main complications were severe, intractable cholangitis, short bowel syndrome with malnutrition; TPN-associated liver injury; and wound problems. Two patients died of ascending cholangitis, 1 patient of liver failure that was exacerbated by TPN-associated liver injury, and 1 patient is awaiting a liver transplant. Only 1 patient is in good health, being anicteric and showing normal growth and development. CONCLUSIONS Biliary atresia is evidently closely associated with meconium peritonitis caused by perforation of small bowel atresia. The management of these patients is more difficult than that of patients with the usual form of biliary atresia, because of the necessity for a long period of TPN and the combined short bowel syndrome. The ideal management of these conditions has yet to be determined.