Management of extreme short bowel syndrome (SBS) has changed dramatically over the last 20 years with notable improvements in survival and quality of life in patients with this syndrome. A review of our institution's medical records over a 12-year period (1980-1992) revealed 32 patients with < 100 cm (range, 14-94; median, 40) of functional small bowel after intestinal resection. The causes of intestinal loss included necrotizing enterocolitis (11 cases), atresias (8 cases), long-segment Hirschsprung's disease (5 cases), midgut volvulus (5 cases) and gastroschisis (3 cases). The mean follow-up period was 4.2 years, and four deaths were recorded (12.5%). Survival of eight of nine (88.9%) patients without an ileocecal valve (ICV) and with < 40 cm of small bowel was noted. The absence of an ICV, however, was associated with significantly prolonged total parenteral nutrition. Follow-up surgical procedures, including intestinal lengthening, tapering enteroplasty. Martin's procedure, longitudinal myectomy-myotomy, and ostomy take-down, were performed in 20 of the patients (64%). Prolonged survival and normal development can be expected for the patient with severe SBS who is given meticulous nutritional support and treated with carefully planned secondary surgical intervention. These results are also seen in patients with extreme SBS (< 40 cm residual small bowel length) and no ICV.