Anal sphincter replacement is a treatment option for severe anal incontinence that is not amenable to direct repair. We describe the unusual case of a 25-year-old male who has been successfully treated by implantable artificial sphincter for severe anal incontinence caused by imperforate anus and concomitant short-bowel syndrome. In early life, the patient underwent several surgical procedures, including a perineal colostomy for a high imperforate anus associated with a rectourethral fistula. At aged 21 years, he underwent a subtotal small-bowel resection and a restorative jejunotransverse anastomosis for acute intestinal necrosis related to intestinal malrotation with volvulus. The length of remnant jejunum was 90 cm. Consequently. the patient became permanently incontinent and required nutritional therapy. An artificial sphincter, after the creation of colonic reservoir, was implanted around the pulled-through colon. At two-year follow-up after implantation, despite short remnant bowel, the patient was fully continent without medication. Normal nutritional status was maintained under 100 percent oral nutrition. The patient's quality of life improved dramatically. He has returned to work and had no major restriction in his level of social functioning. Our case illustrates for the first time the original concept of artificial anal artificial sphincter implantation around a pulled-through colon, which constitutes a different situation from disease with rectum in place.