Ganglioneuromatosis is an infrequent disease characterized by mienteric plexus and enteric nervous fiber hyperplasia, habitually manifested by constipation, pain and abdominal distension. There are two ways of presentation: mucosa or transmural. Regularly may be associated with another pathologies like multiple endocrine neoplasia type 2 and Von Recklinghausen's disease. The objective of this publication is to present an extremely infrequent pathology and make a bibliographic review of its clinical, diagnostic, prognostic and therapeutical aspects. We present a fifty-six year old male with a six month history ofabdominal pain and pseudo-obstruction syndrome. During the hospitalization he presents infectious complications, with septic shock secondary to nosocomial pneumonia and death. A Necropsy was done and ganglioneuromatosis was diagnosed. Due to the low incidence of this pathology, it is rarely included in the differential diagnosis of pseudo-obstructive syndrome in adults. The post-mortem diagnosis moved us to perform a bibliographic search to amplify our knowledge and compare this particular case with those previously communicated by other authors.