BACKGROUND Gangliocytic paraganglioma is an extremely rare tumor, with only 263 reported cases. This tumor has heterogeneous clinical presentation, with gastrointestinal bleeding being the most common. However, jaundice is a relatively unusual presentation, seen in less than 5% of all cases. CASE REPORT We report the case of a 32-year-old man who presented with abdominal pain and jaundice. He reported having similar episodes of this pain recently, but they were milder in severity. On examination, there was a tenderness in the right upper quadrant with a positive Murphy sign. Laboratory investigation revealed total bilirubin of 3.6 mg/dL with a direct bilirubin of 3.0 mg/dL, alkaline phosphatase of 323 IU/L, and g-glutamyltransferase level of 1153 IU/L, giving the impression of obstructive jaundice. The abdominal ultrasound examination revealed a normal common bile duct diameter with no thickening or pericholecystic fluid noted. Subsequently, the patient underwent endoscopic retrograde cholangiopancreatography, which revealed a mass in the second part of the duodenum. Histopathological examination of biopsy specimens obtained by fine-needle biopsy revealed an unencapsulated submucosal lesion with epithelioid, spindle, and ganglion cells. The spindle cells expressed positive immunohistochemical staining for S100, synaptophysin, and chromogranin. These findings were consistent with the diagnosis of gangliocytic paraganglioma. Surgical resection of the tumor was advised. However, the patient refused the operation despite the recommendation of the oncology team. CONCLUSIONS Gangliocytic paraganglioma is a very rare tumor that may present with a clinical picture mimicking a biliary disease. Clinicians should have a high index of suspicion for duodenal lesions in patients presenting with obstructive jaundice with no evidence of biliary stones.