Chronic intestinal pseudo-obstruction (CIPO) is today a significant indication for home parenteral nutrition (HPN) in both adults and children. CIPO refers to a heterogeneous group of disorders characterized by symptoms of intestinal obstruction in the absence of mechanical evidence of obstruction. It is caused by ineffective intestinal contractions. CIPO may be classified either as a primary disease, which is usually limited to the hollow viscera, or as a secondary disease, which is associated with an existing systemic disorder. CIPO may predominate as a “total” gut disease from esophagus to anal sphincter or as a “localized” disease, which is gastric and intestinal or intestinal alone; with the exception of an isolated megaduodenum, segmental gut disease is not a feature. Recurrent episodes of obstruction are the usual clinical presentation with, most frequently, malabsorptive diarrhea; pseudo-pseudo CIPO may also be a manifestation, ie, malabsorptive diarrhea without obvious obstruction. Besides nutritional support, symptomatic treatment usually consists of prokinetic drugs, such as low-dose octreotide or erythromycin. In the case of a systemic disease causing CIPO, specific treatment directed against the primary disease process is another cornerstone of management, for example, in systemic lupus erythematosus. The most severe forms may require ostomies for provision of nutrition or decompression. Other surgical interventions are best avoided, with the exception of those required for such complications of CIPO as peritonitis caused by perforation (with or without diverticula) or bowel ischemia. Patients with the most severe forms of CIPO and, especially, those affected by the diffuse forms of the disease, and who have, in effect, a “functional” intestinal failure, may need long-term HPN. In the latter case, when there is a failure of HPN, alternative treatments, such as extensive resection or intestinal transplantation, should be discussed on a case-by-case basis in a intestinal tertiary care center.