Two patients with neck-tongue syndrome, a rare disorder of the upper cervical nerves that results in paroxysmal neck pain and paresthesia of the tongue, are described. Signs are the result of compression of the C2 root by disorders affecting the first two cervical vertebrae. Conservative management is effective in most cases if no masses are in the involved area. The disorder has been observed rarely in children. Clinical onset occurred at 6 and 11 years of age, respectively, in the reported patients.