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Autonomic symptoms are not uncommon in chronic inflammatory demyelinating polyneuropathy (CIDP), but they are mostly mild and transient and are overshadowed by somatic manifestations. Here, we report a very unusual case of CIDP with severe autonomic symptom, intestinal obstruction, as initial and persistent symptom which responded well to high-dose glucocorticoid and intravenous immunoglobulin treatment.We described a patient with CIDP with precedent and long-lasting incomplete intestinal obstruction. Clinical manifestations were precedent and chronic abdominal pain, distension and constipation, and later numbness and weakness of lower and upper limbs. Radiograph showed incomplete intestinal obstruction, cerebrospinal fluid (CSF) showed albuminocytological dissociation and electromyography indicated neurogenic lesion.CIDP with incomplete intestinal obstruction was diagnosed based on the history, related symptoms, typical abdominal radiograph, CSF albuminocytological dissociation, and electromyographic findings.The patient was treated with intravenous methylprednisolone and immunoglobulin.After treatment, the intestinal obstruction disappeared and the somato-symptoms improved greatly and gradually.This case highlights the need for diagnostic vigilance in cases of incomplete intestinal obstruction of unknown cause. We recommend CSF and electromyography examination in view of rare but possibility of CIDP.