Hirschsprung disease and chronic intestinal pseudo-obstruction (CIPO) in children are the most common and severe disorders of gastro-intestinal tract motility. While the problems connected with the Hirschsprung disease widely coverage in native literature, the publications that devoted the CIPO absent. Aim of the study was to analyze the literature data that devoted to the diagnosis and treatment of children with CIPO. Intestinal pseudo-obstruction refers to heterogeneous groups of disorders with a similar phenotypic presentation characterized by obstructive intestinal symptoms in the absence of true anatomical obstruction. CIPO classification based on the primary dysfunction: affected enteral nervous system (neuropathy), affected smooth muscle (myopathy) or interstitial cells Cajal (mesenchymopathy). Besides that, the intestinal pseudo-obstruction is divided into primary and secondary, congenital and acquired, total and segmentary. The main clinical features, methods of investigation in suspected CIPO such as radiological (roentgenography and roentgenoscopy, computed tomography, cine magnetic resonance), endoscopic, manometry, pathohistological, etc. were listed. The main principles of nutrition support, conventional therapy, and surgical management were presented. Conclusions. CIPO is a severe disorder of gastrointestinal motility that caused by various factors, which can be congenital and/or acquired. Diagnosis of CIPO based on the results of clinical, instrumental, and pathohistological investigation. The management of children with CIPO should include nutritional support, conventional therapy, and, for the clear indices, surgical correction. No conflict of interests was declared by the authors.