A 66-Year-old man presented with diplopia, headache and dizziness. Magnetic resonance imaging showed a right intrasphenoidal paracavernous mass. A biopsy was performed by endonasal route in the lesion which appeared to be part of the hypophyseal fossa. Histological examination revealed a highly cellular tumoral proliferation, with a variable architecture, of which chief cells were immunoreactive for neuroendocrine markers and spindle-shaped cells positive for PS 100. Morphological features were consistent with the diagnosis of paraganglioma. Our observation is the first case of this rare tumor reported in the French literature.