We report a patient with chronic inflammatory demyelinating polyradiculoneuropathy and progressive resistance to standard treatment who showed a striking response to methotrexate, 20 mg/week. The improvement, which started 5 months after initiation of therapy, was consistent and permanent. It allowed the previously wheelchair-dependent patient to achieve pharmacological remission. The 2-year follow-up of this case further illustrates the role that methotrexate may play as a treatment option for CIDP patients.