Anorectal atresia, which is classified as a low anorectal malformation, is characterised by the absence of the anal verge and by variable rectal atresia. In some cases, which have been classified as rectal agenesis, the atresia is associated with the absence of the internal sphincter. The therapeutic options are definitely surgical, aiming to relieve the bowel occlusion and to restore faecal continence by lowering the cul-de-sac to the perineum. We present the case of an adult patient with congenital rectal agenesis, double fistula (cul-de-sac-urethra and cul-de-sac-perineum) and caecostomy since birth. The patient was treated with a resection of sigmoid-rectum for the presence of a 20-cm faecaloma in the cul-de-sac, with a non-continent pull-through, and with implantation of an artificial bowel sphincter. Despite some difficulties in managing the device and a slight symptomatic mucosal prolapse, the results after 30 months have so far satisfied both the patient and the medical staff, especially in consideration of the limited number of alternative therapies.