Angiocentric glioma is a rare brain tumor commonly found in frontal or temporal lobes. It has a benign course, and surgical resection can be curative. Brainstem location is extremely rare, with only six cases reported so far in the literature. In the present study, the seventh case of brainstem angiocentric glioma has been reported, and its course in comparison with supratentorial location and the role of molecular diagnosis has been discussed.