Trigeminal neuralgia is a very peculiar disease. The pain, also known as "tic douloureux", is paroxystic and very severe. It can be triggered by a light cutaneous stimulus on a very localized spot on the face (the so-called "trigger zone"). The patient can sometimes benefit from long remissions without any treatment. With the exception of multiple sclerosis and of uncommon cases of posterior fossa tumours or other lesions impinging on the trigeminal nerve, ganglion or root, trigeminal neuralgia is considered as "idiopathic". Some benign abnormality had for long been suspected. The current opinion is now in favour of a "neurovascular conflict": an artery, most often a loop of the superior or anteroinferior cerebellar artery, has an offending contact with the trigeminal nerve root, which results in localized demyelination and ectopic triggering of neuronal discharges. This hypothesis is in agreement with the relief provided by antiepileptic drugs and is supported by recent neuroimaging data. Therapeutic options are reviewed: very efficient drugs are available but fail to provide a significant relief and/or have important side effects in many cases. Surgical alternatives are available, for which guidelines are proposed.