Purpose To investigate clinicopathological features, immunohistochemical findings and differential diagnosis of papillary glioneuronal tumor ( PGNT) . Methods The clinical features, imaging, histological and immunohistochemical findings were analyzed in three cases of PGNT, along with review of the related literatures. Results Two patients were female adults, and the other was male adults. One presented with dizziness, and the other exhibited intractable seizures. Magnetic resonance imaging ( MRI) revealed a cyst-ic space-occupying mass in brain parenchyma of left temporal lobe or right parietal lobe. Microscopically, it was characterized by pseu-dopapillary structures composed of hyalinized vessels rimmed by cuboidal glial cells, oligodendrocyte-like cells and the proliferation of neuronal cells. On immunohistochemical staining, the perivascular cuboidal cells were GFAP-positive, the oligodendrocyte-like cells were Olig-2-positive, and the neuronal cells were synaptophysin-and nuclear protein-positive. Conclusion PGNT is a rare and new variant of mixed neuronal-glial neoplasm, the natural evolution of this tumor is one of low malignant potential and it has a good progno-sis. The distinctive pathologic features help to differentiate this entity from other neoplasm of the central nervous system which has pa-pillary structures.