BACKGROUND: Essential erythermalgia is a rare acrosyndrome that is difficult to treat. Herein, we report a new case unusual in terms of both the associated partial epileptic seizures and of the favourable outcome achieved through antiepileptic treatment with oxcarbazepine. PATIENTS AND METHODS: A male adolescent born of a non-consanguineous marriage had been followed since the age of 5 years for essential erythermalgia. A number of different treatments had been tried in succession but without success: beta blockers, acetylsalicylic acid, paracetamol and indomethacin. At the age of 15 years, the patient presented serious depression requiring antidepressant therapy with sertraline. Seven days after the start of this medication, the patient presented temporal partial epileptic seizures confirmed by electroencephalogram. The antidepressant was stopped and a new-generation antiepileptic, oxcarbazepine, was instituted. The epileptic seizures subsided and so too did the paroxysmal pain associated with erythermalgia, in addition to which the patient's skin lesions improved. This therapeutic efficacy has persisted for 21 months. DISCUSSION: The novelty of this case report concerns the association of essential erythermalgia and epilepsy and the favourable outcome achieved with antiepileptic therapy. It may well be that the patient's erythermalgia and epilepsy were both responsive to sodium channel therapy, as described with gene SCN9A disorders; however, this point could not be checked in the present case. In practice, we feel there are grounds for use of oxcarbazepine as an alternative treatment in the management of essential erythermalgia.