Primary paraganglioma of the gallbladder is an extremely rare pathology. Only 21 cases have been reported in the literature, and we present the first reported case of gallbladder paraganglioma in a patient with inflammatory bowel disease (IBD) and primary sclerosing cholangitis. A 54-year-old lady with history of chronic right upper quadrant pain on a background of inflammatory bowel disease and primary sclerosing cholangitis underwent laparoscopic cholecystectomy due to gallbladder polyps and gallbladder wall irregularity on surveillance imaging. Her histopathology incidentally noted gallbladder paraganglioma. Due to their scarcity, there is no consensus on management and follow-up of gallbladder paraganglioma. All previously reported cases have been thus far benign and adequately treated with cholecystectomy, however, intra-abdominal extra-adrenal paragangliomas have malignant and metastatic potential hence we would advocate for surveillance.