We describe a case of Restless Legs Syndrome (RLS) associated to periodic limb movements (PLMs) in a woman with a cervical schwannoma. Neurological examination, laboratory tests, neurography and electromyography were unremarkable. Neuroimaging evidenced a schwannoma at C3 level that compressed the right ventral surface of the medulla. Somatosensory-evoked potentials showed absence of the N13 after right median nerve stimulation and reduction of amplitude of the same component after left nerve stimulation. A video-polysomnography documented PLMs with a marked prevalence of the right-sided movements. We believe that the cervical schwannoma played a role in the pathogenesis of RLS and of lateralized PLMs.