Although atypical teratoid rhabdoid tumours preferentially arise in the posterior fossa of infants, we encountered a 56 year old woman with an atypical teratoid rhabdoid tumour located in the sella. She presented with right abducent and oculomotor nerve paresis. Magnetic resonance imaging demonstrated an intrasellar tumour impinging on the right cavernous sinus. Microscopically, the tumour was composed of cells with rhabdoid features; we observed atypia, eccentric nuclei, and intracytoplasmic inclusion bodies. The Ki-67 labeling index was around 30%. The tumour cells were positive for vimentin, epithelial membrane antigen, and neurofilament, but negative for INI1. Despite extended local brain and whole-spine irradiation she died of neural axis dissemination.