Gallbladder paraganglioma (GP) is a rare tumor, with only 12 cases reported in the literature to date. Due to its rarity, clinical information of GP is insufficient. We present a case of GP in a 48-year-old female along with a literature review of all GP cases described to date. A 48-year-old female presented with intermittent right upper abdominal pain. Preoperative imaging revealed a hematoma in the gallbladder lumen without any definite etiology. Laparoscopic cholecystectomy was performed. Gross examination of the gallbladder revealed multiple small stones and a large hematoma as well as a 1.6-cm-sized polypoid mass at the gallbladder fundus. Microscopic study of the polypoid mass showed a zellballen appearance. Immunohistochemical analysis revealed that the mass was positive for synaptophysin, CD56, and chromogranin, suggesting GP. GP is difficult to diagnose because of non-specific clinical findings. Almost all GP cases are diagnosed based on histologic findings after cholecystectomy. Simple cholecystectomy was performed as a treatment in all reported cases of GP, including our case. There was no postoperative tumor recurrence or metastasis after surgery.