Chronic intestinal pseudo-obstruction (CIPO) is a syndrome associating chronic or recurrent obstructive symptoms with intestinal dilation on imaging but without organic obstruction in the digestive tract. It is a rare disease with varying severity whose diagnosis is very complex. The diagnosis is based on clinical and paraclinical arguments in the context of repetitive occlusive syndromes when no mechanical obstruction of the digestive lumen is observed. Abdomino-pelvic computerized tomography (CT) must be performed to rule out a mechanical obstruction. An additional reference examination is trans-duodenal manometry of the small intestine, which is almost never normal in CIPO, but the test is rarely systematically performed. CIPO can be primary (acquired or congenital) or secondary to a systemic pathology (neurological, metabolic, etc.) resulting in neuromuscular damage to the intestinal tract. There are familial forms associated with genetic mutations. The majority of CIPO cases are idiopathic. Symptoms of the CIPO syndrome should be investigated with a complete assessment, guided by questioning and clinical examination that should also focus on urinary, neurological and cardiac involvement. Pathological tissue analysis is interesting for the etiological classification but is difficult to obtain. CIPO must be distinguished from non-CIPO intestinal dysmotility. Management must be carried out in an expert center with multidisciplinary care involving gastroenterologists, nutritionists, psychologists, radiologists, pathologists and digestive surgeons. It is essentially based on symptomatic management (especially with pro-kinetic agents and analgesics), nutritional support, as well as psychological support in view of its impact on quality of life. Surgical management is sometimes necessary.