Gangliocytic paraganglioma (GP) is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. We present a case of duodenal GP treated first by local resection followed by pancreaticoduodenectomy. Frozen section examination of the first specimen was diagnosed as low grade mesenchymal tumor. Formalin fixed paraffin section histology revealed composite tumor composed of three characteristic histologic components: epithelioid, ganglion, and spindle cell. Immunohistochemistry tumor cells were positive for neuron specific enolase, synaptophysin, chromgranin, S100, and neurofilament protein. Cytokeratin and bcl-2 were focally positive. Single mitotic figure was noted. Ki67 labeling index was < 3–4%. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor in the periampullary area. We discuss the differential diagnosis and review the current published literature on GP.