We report three patients with isolated unilateral hypoglossal nerve palsy who experienced an excellent outcome. In two patients no cause was found. Our study seems to confirm that the occurrence of benign and idiopathic isolated unilateral palsy of the hypoglossal nerve is more frequent than previously reported. We would like to stress that neuroimaging studies remain mandatory in order to exclude other common causes, such as tumour and spontaneous or traumatic vascular lesions, in which a specific treatment is necessary.