The aim of the study was to evaluate the long-term effects of neonatal short bowel syndrome on cognitive functions during development. Nine patients diagnosed with short bowel syndrome during the neonatal period were enrolled in this study. Their medical records were reviewed; anthropometric measurements and blood tests were assayed; IQ tests (the Chinese versions of WAIS-R, WPPSI-R and WISC-R) were performed depending on their age, and a BSID assessment was carried out in those patients less than 4 years old. Eight of 9 patients were followed up except for one patient who died in a car accident at the age of three. All patients had been weaned off parenteral nutrition for more than 2 years. The average residual small bowel length was 58.1 cm (range 35-70 cm), and the mean parenteral nutrition (PN) duration was 73.1 days (43-147 days). The mean duration of the period without PN was 7.4 years (range 2.1-17.1 years). Weight, height and BMI for age were normal in 7 children except for 1 child, who was overweight. Hemoglobin and albumin concentrations were normal in all 8 patients. Evaluation of cognitive development showed normal results for all 8 patients while a verbal/performance discrepancy was found in 2. Patients with neonatal SBS who were weaned off PN for more than 2 years were found to have normal growth and cognitive development during this long-term follow-up. There was no evidence for a strong correlation between SBS and nutritional/cognition disorder. Longer term and controlled studies with a larger sample size are warranted.