Chronic intestinal pseudo-obstruction (CIPO) is a rare and life-threatening digestive disorder characterized by significantly reduced intestinal peristalsis, leading to the pathological intestinal dilatation and persistent obstructive symptoms without any mechanical cause. Etiologically, CIPO is classified into idiopathic and secondary. In cases with secondary CIPO, systemic sclerosis is the most common cause. Medication is the primary treatment; however, management is generally difficult. In severe cases, nutritional therapy and decompression therapy play a key role. Percutaneous endoscopic gastrojejunostomy has recently been in the spotlight, in that this method allows for on-demand decompression at home without nasal tube insertion, reducing patients’ burden and improving quality of life. Conversely, intestinal resection should be avoided, except in emergencies such as strangulation, because it often causes postoperative dilatation of the small bowel and further progression of malnutrition due to short bowel syndrome. Prognosis is generally poor, with a 5-year survival rate of 78% and a 10-year survival rate of 75% in adult cases requiring home intravenous hyperalimentation, extremely low for a non-malignant disease. Better prognosis is reported for cases with preserved oral intake and younger onset (under 20 years), while secondary CIPO with systemic sclerosis has a poorer prognosis. Appropriate management is crucial, and recognition of this disease is essential.