The clinical data of two male patients with duodenal ganglion paraganglioma (GP) were collected and clinicopathological researched. HE staining and immunohistochemical staining were used to observe the pathological changes of the surgical specimens. The clinical and pathological features were analyzed by review the relevant cases reported in the literature. There are 3 cell components seen under the microscope: neuroendocrine cells, spindle cells and ganglion cells. Epithelioid cells arranged in nests or trabecular patterns; spindle cells were distributed in bundles or sheet patterns between nests of epithelioid cells; the ganglion cells scattered in a single or between these two types of cells. Immunohistochemically, the epithelioid cells were positive for CgA, Syn; the spindle cells were positive for Vimentin, and the ganglion cells were positive for CgA, Syn and S-100. GP is a rare tumor which is commonly located in the duodenum. The correct diagnosis can be made according to the specific pathological characteristics of the microscopic performance and immunohistochemistry.