Chronic intestinal pseudo-obstruction is a severe syndrome characterized by a profound derangement of the intestinal propulsive motility that resembles mechanical obstruction, in the absence of any mechanical obstruction. This syndrome represents one of the main causes of intestinal failure and is characterized by impairment of physical growth and development as well as by a high rate of morbidity and mortality. It may be idiopathic or secondary to a variety of diseases. Most cases are sporadic, even though familial forms with either dominant or recessive autosomal inheritance have been described. Based on histological features intestinal pseudo-obstruction is classified into 3 main groups: neuropathies, mesenchymopathies, and myopathies, according to the predominant involvement of enteric neurones, interstitial cells of Cajal, and smooth muscle cells, respectively. Treatment of intestinal pseudo-obstruction involves nutritional, pharmacological, and surgical therapies, but it is often frustrating and does not change the natural course in the majority of cases. The nutritional management has a crucial importance in pediatric age and involves the administration of special formulae, the enteral delivery of nutrients, by a nasogastric tube, percutaneous gastrostomy, or jejunostomy. In the most severe cases, parenteral nutrition becomes mandatory in order to satisfy nutritional requirements and manage appropriately obstructive episodes.