The clinical data of a 42-year-old male patient with duodenal ganglion paraganglioma were collected. 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 at home and abroad. There are 3 cell components seen under the microscope: neuroendocrine cells, spindle cells with Schwann cell-like differentiation (spindle cells) and ganglion cells. The neuroendocrine cells were arranged in pseudo glandular tubular structure, eosinophilic cytoplasm, with the same size of the oval nucleus and without nuclear fission. The spindle cells formed small bundles, wrapped in neuroendocrine cells; the ganglion cells are scattered distribution; there was no obvious cell atypia in the 3 kinds of cells. Wispy smooth muscle component was seen in this case, too. Immunohistochemistry showed neuroendocrine cells were positive for CgA, Syn,CD56 and CK, spindle cells were positive for S-100 and vimentin, Ganglion cells were positive for S-100 and NF. Duodenal ganglion paraganglioma is a rare tumor and each part of duodenal may occur. The correct diagnosis can be made according to the characteristics of the microscopic performance and immunohistochemistry.