The paired box 3 (PAX3), a crucial transcription factor, is normally expressed during embryonic development and is absent in normal adult human tissues. Deregulated expression of PAX3 has been observed in tumors like rhabdomyosarcoma and melanomas. To assess deregulated PAX3 expression in patients with gliomas, these samples from 57 glioma patients (13 grade I, 16 grade II, 14 grade III, and 14 grade IV tumors) and 10 normal brain specimens acquired from 10 patients undergoing surgery for epilepsy as control were obtained. PAX3 expression was measured by RT-PCR, Western blot, and immunohistochemistry. Survival analyses were performed using the Kaplan-Meier method. Association between PAX3 expression, clinicopathological characteristics, and patients' survival were analyzed by using SPSS 17.0. We found that the expression of PAX3 was upregulated in high-grade glioma tissues compared with that in low-grade and normal brain tissues, and increased with ascending tumor World Health Organization (WHO) grades (P = 0.001). The increased PAX3 expression in gliomas was significantly associated with higher WHO grade (P = 0.021) and poorer disease-specific survival of patients (P = 0.001). Our results suggested that PAX3 might be an intrinsic regulator of progression in glioma cells and it might serve as a prognostic factor for this dismal disease.