Tumours of the glomus jugulare always raise diagnostic and therapeutic problems. We decided to review the pathology and clinical findings whilst reporting a typical case found on our neurology unit. After a brief review of the embryology, anatomy and histology of the glomus jugulare, the authors report the case of a patient who presented a recurrent intracranial form with involvement of several cranial nerves, together with associated involvement of the carotid glomus. This was a familial disease. The patient was operated on with excellent results. The authors then present a clinical description of the various signs in glomus tumours,; depending more on the origin of the tumours than on the length of history. They then criticize the diagnostic interest of radiology, emphasizing the interest of angiography which may demonstrate a pathological vascular focus derived from the internal carotid artery together with the vertebro-basilar system. Retrograde jugulography also provides important information. The treatment of glomus jugulare tumours is, above all, surgical. They are approached through the ear in order to localise the intra-petrous part, or else, they may be approached through the skull. A combined otological and neuro-surgical approach to sometimes necessary for the lesion may involve both the petromastoid region and the posterior cerebral fossa. Radiotherapy is often advised after operation, in view of the importance of the vascular component of these tumours. The results of treatment are, on the whole, satisfactory.