A 76-year-old woman presented with an intrasellar and suprasellar mass which caused deterioration of visual acuity and bitemporal visual field defects. Transsphenoidal and transcranial partial resection revealed a primary chemodectoma. This tumour is very rare in the sellar area, where there are normally no paraganglionic cells. We review the literature and discuss possible mechanisms for the development of this tumour.