A 9-year-old male child with sickle cell disease presented with abdominal pain of four days and bilious vomiting for one day. The urine was dark and the stool was pale. Upon admission, the patient was sick and deeply jaundiced. The vital signs were stable but for a low-grade fever. The abdomen showed generalized tenderness, especially in the epigastrium and right hypochondrium. There was no organomegally. The complete blood count showed leukocytosis and reticulcytosis (Table 1). Serum electrolytes were normal. The liver function tests showed an elevated total bilirubin (TB) and direct bilirubin (DB). Alkaline phosphatase was elevated; serum amylase and liver transaminases were within normal limits. The coagulation profile showed a slight elevation versus control. The plain abdominal x-rays were normal Hemoglobin electrophoresis showed a hemoglobin-S of 90%. Abdominal ultrasonography (US) showed a contracted gallbladder with no evidence of acute inflammation, but with very doubtful small stones or sludge within. There was mild intrahepatic biliary dilatation. However, the CBD was not dilated and did not contain any stones. No free intra-abdominal fluid was seen. The spleen was not visualized. The patient was treated conservatively in the hope that a small CBD stone or sludge would pass. The patient improved within a few days. Blood culture showed no growth. Hepatitis serolog and HIV tests were negative. Ten days later, symptoms recurred. The patient became febrile (38.5 C), and total and direct bilirubin increased to 50 and 32 mg/dL, respectively. Serum amylase remained normal. Repeated abdominal U.S showed normal gallbladder and CBD but with free fluid in the peritoneal cavity. A computed tomography was normal apart from the free fluid in the peritoneal cavity and an absent spleen. Endoscopic retrograde cholangiopancreatography (ERCP) showed free flow of bile into the duodenum, no stones in the CBD and no leakage of the dye through the biliary tree. ERCP was not performed on first admission because the patient was seen in the pediatric medical service at that time. The patient improved clinically and his total and direct bilirubin declined to 18 and 12 mg/dL, respectively. Five days later, the patient became very sick with more severe symptoms and signs. The abdomen became distended and tender all over with rigidity and rebound tenderness. The patient was optimized for surgery. By laparotomy, about 500 mL of bilious ascetic fluid was drained. Operative cholecystogram showed multiple small stones in the gallbladder (GB) and distal portion of the CBD. A cholecystectomy was done and the CBD explored. Multiple small pigment stones were removed with saline irrigation. A sealing perforation was found in the mid-CHD. A T-tube (T-T) with a long proximal limb was inserted to stint the perforation site and the perforation was repaired with absorbable sutures. An omental patch was used to support the repair site. A repeat operative T-tube cholangiogram showed no other leaks. A tube drain was left in the subhepatic region. The patient improved clinically and his total and direct bilirubin declined to 7.2 and 5 mg/dL, respectively. There was leak of bile around the T-tube, which started one week postoperatively. Blood culture grew Enterobacter species, which were treated by antibiotic. A T-tube cholangiogram 20 days postoperatively showed minimal leak from the CBD and no residual stones with free flow of dye to the duodenum. The T-tube was later removed followed by tube drainage three days later. The patient was discharged in good health.