We report a case of extranodal NK/T-cell lymphoma, nasal type, with exclusive cerebral localization in a patient with AIDS. The patient presented with neurological alterations, fever and convulsions, so the initial presumptive diagnosis was an opportunistic brain infection. MRI showed a left parietal necrotic lesion and a stereotactic brain biopsy was performed for pathological, microbiological and molecular studies. Histological sections showed an angiocentric and angiodestructive growth pattern and the immunophenotype of this tumor was CD56+, CD45+, CD3+ (cytoplasmic), Granzyme B+ and Perforin+. All the microbiological studies such as bacterial, fungi, micobacteria, Toxoplasma gondii and Cryptococcus determination were negatives. A PCR study with primer specific for EBV viral genome of Bam-Hi-w system was positive. Also, a rearrangement study showed T-cell gene rearrangement with monoclonal appearance. A diagnosis of extranodal NK/T-cell lymphoma was made and the patient died a few days later. This case represents a very rare example of NK/T-cell lymphoma of the brain in a patient with AIDS. The diagnosis of this kind of lymphomas requires a multimodality approach correlating clinical, morphological, immunophenotypic and molecular data.