Chronic intestinal pseudo-obstruction is a rare syndrome characterized by recurrent episodes of small bowel obstruction without evidence of a structural obstructing lesion. The two pathophysiologic types of this motility disorder are myopathic and neuropathic. The latter may affect extrinsic or intrinsic neural control of gut motility. Diagnosis is based on (1) recognition of the clinical syndrome and exclusion of mechanical obstruction by endoscopy, radiologic studies, or laparotomy and (2) manometric studies of the stomach and small bowel. Full-thickness biopsy specimens for histologic analysis may not be essential for the diagnosis in the future. The goals of treatment are the restoration of normal gut peristalsis and the correction of nutritional deficiencies. Prokinetic medications, surgical excision in cases of localized disease, and parenteral nutrition are frequently necessary. Management is difficult because of the lack of efficacious medications, extension of the disease to other regions, and complications of central parenteral nutrition. Prokinetic agents, venting enterostomies for relief of symptoms, and enteral supplementation are being evaluated in this intractable and serious condition.