Purpose: We present a rare case of a gangliocytic paraganglioma (GP) in the common bile duct causing painless, obstructive jaundice. Methods: Case Report Results: A 71 year old female presented to the emergency room with a history of fluctuating jaundice with loose bowel movements for 8 weeks. Initial evaluation several weeks prior with ultrasound and abdominal CT were unremarkable. She denied most other symptoms including fever, chills, pain and bleeding. She did have a past history of breast cancer treated with lumpectomy and radiation. Physical examination showed scleral icterus and jaundice, but a benign abdominal exam. Laboratories were significant for a mild elevation in the CA 19-9 (114), normal CEA, total bilirubin of 11.5, Alkaline Phosphatase 42, INR 1.92, Albumin 1.8. A repeat ultrasound revealed evidence of a dilated common bile duct (12 mm) and intrahepatic biliary dilation. The patient underwent an endoscopic retrograde cholangiopancreatogram which revealed a 2 × 3 cm polypoid mass involving the distal common bile duct just above the intrapancreatic portion approximately 2–3 cm proximal to the ampulla. Brushing was performed for cytology, but this returned negative. A sphincterotomy with billiary stent placement was performed. The patient's jaundice improved substantially subsequent to this. CT and MR – cholangiogram of the abdomen showed a smooth soft tissue density in the common bile duct. There was still high suspicion for malignancy, so she then underwent an exploratory laparotomy. Cholecystectomy, common bile duct exploration and resection of the intra and extrahepatic common bile duct were performed, along with regional portal lymphadenectomy and a Roux-en-y choledochoduodenostomy. The specimen did show evidence of acute and chronic cholecystitis. The common bile duct revealed a 2.5 × 2 × 3 cm ulcerated polypoid mass. Microsocpic examination showed nests of ganglionic cells with enlarged densely hyperchromatic nuclei, some of which were multi-loculated, all compatible with gangliocytic paraganglioma. All other pathology specimens revealed no evidence of tumor. On follow up two months and eight months after surgery, the patient was doing well, without any further jaundice or symptoms. Conclusion: GPs are rare tumors first described in 1962. The duodenum is the most common site for GPs. Gastrointestinal bleeding and abdominal pain are common presenting symptoms. Its usual endoscopic appearance is as a submucosal mass, often resulting in negative mucosal biopsies. These neoplasms are largely benign in clinical course, although a few cases of regional lymph node metastases have been noted. A review of the literature shows 4 cases similar to ours where the GP originated in the biliary system.