Chronic intestinal pseudo‐obstruction (CIPO) is a rare pathological condition characterized by a marked derangement of gut propulsive motility mimicking mechanical obstruction, in the absence of any lesion occluding the gut lumen. This disease is often associated with a disabling and potentially life‐threatening complications and is still too often unrecognized even in referral centres. As a result, patients receive neither appropriate care nor recognition of their severe health condition. Medical and surgical therapies are often unsatisfactory and long‐term outcome turns out to be poor in the vast majority of cases. This article focuses on the main clinical features, the management and long‐term outcome of patients affected by CIPO, with particular emphasis on those aspects which remain a matter of debate.