Pseudo-obstruction of the gastrointestinal tract is a rare disorder of impaired gastrointestinal motility. The more common symptoms of pseudo-obstruction in the infant or child include dysphagia, nausea, vomiting, abdominal distention, abdominal pain, and constipation. The majority of children have symptoms within the first year of life. Chronic cases of pseudo-obstruction are associated with neuropathic or myopathic changes in other parts of the body. Bladder dysfunction and neurological problems have been reported. The diagnosis of pseudoobstruction is based on history, physical examination, radiographic studies and motility studies. Advances in medical technology have facilitated the identification of abnormal motility patterns in children. Therapy for pseudo-obstruction is primarily supportive. The use of motility agents has been unsuccessful in treating pseudo-obstruction. Nutritional and antibiotic therapy are the mainstays of treatment. Nursing interventions, patient/family education and advances in home care technology have improved the quality of life for children with pseudoobstruction. Small bowel transplantation offers hope for the future.