The term chronic intestinal pseudo-obstruction describes a syndrome of severly altered gastrointestinal motility that clinically resembles mechanical intestinal obstruction. The syndrome comprises numerous underlying primary or secondary neuropathies of the intrinsic or entrinsic nervous system as well myopathies. Almost a third of the patients requires long-term total parenteral nutrition (TPN). However, emergency surgery and even small bowel transplantation as an ultimate option after failure of TPN may become necessary to evade a vital threat. Although our understanding of pathogenesis and therapeutical options is still evolving, current knowledge allows a differentiated diagnostic approach, classification of the primary and secondary causes and differentiated therapy.