Chronic intestinal pseudo-obstruction denotes the clinical picture that results due to the failure of intestinal peristalsis to overcome the normal resistance to flow and is characterized by recurrent episodes of signs and symptoms of intestinal obstruction in the absence of any mechanical compromise of the intestinal lumen. The region(s) of the gut affected may be isolated or diffuse. It is not uncommon to find evidence of autonomic neuropathy and smooth muscle dysfunction with extraintestinal manifestations such as urinary symptoms from abnormal ureter or bladder function. Intestinal pseudo-obstruction can be caused by a variety of diseases, and for simplicity, certain authors have divided it into myopathic and neuropathic categories. Intestinal pseudo-obstruction may present at any age with a variable amount of abdominal pain, distension, nausea, diarrhea, or constipation and with laboratory abnormalities usually reflecting the degree of malabsorption and malnutrition present. The radiologic findings are varied but commonly include paralytic ileus or signs of apparent clinical obstruction with dilated loops of bowel. The number of pseudo-obstruction cases is dependent on how one defines the condition. It appears prudent to require radiographic abnormalities consistent with obstruction on a plain film of the abdomen for the diagnosis. More recently, studies have focused on the gastrointestinal manometric abnormalities of the stomach and small intestine in chronic intestinal pseudo-obstruction during fasting and fed states; however, sensitivity and specificity of these abnormalities are not well defined. Treatment is aimed at limiting symptoms and maintaining adequate nutrition. Prokinetic agents should be tried in an attempt to restore normal intestinal propulsion. However, their overall efficacy appears to be variable. It is still too premature to consider intestinal pacing or small bowel transplantation in this condition. Surgical approaches to chronic intestinal pseudo-obstruction should be limited to patients refractory to medical therapy, and even then, an approach focused on the patient's primary presenting symptoms should be considered.