Papillary glioneuronal tumor (PGNT) has recently been identified as a new variant of mixed neuronal-glial tumors. We report the clinical and pathological features of PGNT in two Chinese patients. One patient was a 35-year-old man who suffered from intractable seizures for 16 years. Another was a 26-year-old woman who presented with headache for 2 years. In both patients, magnetic resonance imaging showed well demarcated, mixed cystic and solid tumor in the temporal lobe. Histology of the excised tumors revealed a pseudopapillary architecture surrounded by a glial component and intervening areas were occupied by neuronally differentiated cells. No cortical dysplasia was found in the neighboring cortex in one of them. The glial component showed immunoreactivity with glial fibrillary acidic protein and S-100 protein. Neuronally differentiated cells were immunolabeled by antisynaptophysin, NF, NeuN and MAP2 antibodies. Some small cells surrounding the surface of the pseudopapillae and in the compact area were immunopositive for Olig2. The MIB-1 labeling index was < 3%. The tumor did not recur within the follow-up periods of 50 months and 13 months, and the patient with temporal lobe epilepsy became seizure-free after surgery.