Intestinal failure (IF) is a term used to define the state where intestine’s function is significantly reduced, to the point where adequate growth and hydration cannot be maintained. In such cases, intravenous nutritional support is essential for sustaining the patient’s life. In pediatric patients, the most common cause of IF is short bowel syndrome (SBS). Due to the prolonged treatment and high complication rates, management of SBS remains a continuous challenge to many physicians. Herein, we report the case of a 2,260 g premature female infant born at 35-week gestational age with type 4 jejunoileal atresia. She presented with ultrashort bowel syndrome, having a bowel length of less than 15 cm, but ultimately achieved gut autonomy and restored bowel function through successful intestinal rehabilitation within the first two years of life.