Paraganglioma is an exceedingly rare tumour of the duodenum that arises in close proximity to the ampulla of Vater. To date a total of 133 cases of duodenal paraganglioma have been reported in the literature; of these, 27 (20%) were histologically gangliocytic paragangliomas. This neoplasm generally behaves in a benign fashion, although instances of recurrence and/or lymph node metastasis have been described. The treatment consists in endoscopic polypectomy or surgical resection in relation to the histological features and the macroscopic extent of the neoplasm. We present a case of a benign duodenal gangliocytic paraganglioma treated by a laparo-endoscopic approach. We report a case of gangliocytic paraganglioma in a 75-year-old woman admitted to the General Surgery Division of Aosta Regional Hospital (Aosta-Italy), complaining of melaena and anaemia. Upper gastrointestinal endoscopy followed by enteroscopy with a video-capsula, revealed a pedunculated neoplasm in the second portion of the duodenum, with ulceration of the overlying mucosa. Multiple biopsies were performed during the endoscopic examination and showed the cellular pattern of benign paraganglioma. After stabilisation of the patient's clinical status, we performed a resection of the neoplasm via a laparoscopic transduodenal approach and a concomitant intraoperative duodenoscopy. The histological features showed a gangliocytic paraganglioma without a malignant cell pattern. The size of the neoplasm was 4 cm. The resection margins were free of neoplastic infiltration. The postoperative stay was 9 days and there were no intraoperative or postoperative complications. The patient is currently in good health without any tumour recurrence. Transduodenal laparoscopic resection with intraoperative duodenoscopy is a valuable treatment for benign gangliocytic paraganglioma of the duodenum which is unresectable by upper gastrointestinal endoscopy. This approach affords the advantages of the minimally invasive technique and fulfils the surgical tenets of the open transduodenal approach, if en bloc resection of the neoplasm with the adjacent duodenal wall is performed.