Background: Gangliocytic paraganglioma of the duodenum is a peculiar neuroendocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology, consisting of endocrine, ganglion and spindle-shaped Schwann cells. Methods: A case of a duodenal gangliocytic paraganglioma is reported in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portion of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography. The tumor was simply excised through a duodenotomy. Results: The size of the tumor was 4.5 × 3 × 1.5 cm. Immunohistochemical and ultrastructural features of the tumor classified it as a typical duodenal gangliocytic paraganglioma with positive cellular reaction for neuron-specific enolase, chromogranin, somatostatin, gastrin and S100. Conclusion: Forty-six months after surgery, the patient is well with no evidence of recurrence. The majority of the reported duodenal gangliocytic paragangliomas were of bening nature. However, the fact that in 4 isolated cases there was lymph node involvement indicates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up.