Objective To investigate the expression of α-thalassemia/mental retardation syndrome X-linked (ATRX) in different central nervous system tumors and the meaningness in differential diagnosis. Methods Expression of ATRX protein was determined by immunohistochemistry in astrocytomas , oligodendrogliomas, glioblastomas, ependymomas and meningiomas. Results Positive expression levels of ATRX in astrocytomas were significantly lower than those in oligodendrogliomas,glioblastomas, ependymomas and meningiomas (all P <0.01). Conclusion The loss of ATRX was mainly occurred in astrocytomas, which could be as a astrocytoma marker in diagnosis.