“Vanishing bowel” has been rarely reported in the literature. This case and discussion are intended to describe the surgical, medical, and nutrition management of this unusual congenital abnormality. Of 3 reported cases surviving vanishing bowel, one had substantial residual small bowel, another had residual small bowel and bowel-lengthening procedure, and one received an intestinal liver transplant. The patient presented in this case had residual small bowel and lengthening procedure. The patient described is well as of October 2005 (age 3.5 years), still receiving nocturnal parenteral nutrition (PN), but is eating solid foods without significant malabsorption or abdominal distention. He has experienced modest weight gain over the past 2 months.