Meningomyelocele (MMC) is usually a nonfatal malformation but combined with significant morbidity and disability. One of the lifelong problems is fecal incontinence caused by lack of sensation, sphincter control, and rectosigmoidal motility. The authors asked whether a fetal repair of MMC may induce normal development of rectal muscle and nerves. In a sheep model, MMC from L 1 to S 2 was created at day 75 of gestation with local removal of skin and muscle, laminectomy, and incision of dura and central canal in 12 animals. Eight animals had MMC repair at 100 days gestation by standard neurosurgical closure, alloderm- or Goretex-coverage. Four animals remained as controls. Cesarean section was done at day 140 of gestation (term, 147 days) and the animals were killed. The rectum was examined with 3-μm slices and stained with H & E for conventional histology and S 100 to analyze nerve fibers and ganglion cells. The 4 untreated MMC sheep showed a hypoplastic longitudinal muscle layer with small fascicles and a low density of myofibrils. The circular muscle layer looked normal. The submucous plexus was hypoplastic as well, whereas the myenteric plexus was unchanged. In all sheep with different intrauterine closures of MMC, all intestinal muscle layers showed normal muscle fibers, large fascicles, and a high density of myofibrils. Both plexes were normal. Fetal surgery for repair of MMC allows for normal development of rectal muscles and nerves. It seems that this surgical intervention can prevent the so-called “second hit” of spinal cord injury by local irritation. A simple coverage with impermeable material is sufficient and shows the same results as with intrauterine neurosurgical repair. This observation raises the possibility for an endoscopic (amnioscopic) procedure that would be less invasive. The results of the study are very encouraging, and pediatric surgeons will be extremely interested in postpartum functional controls, which are still lacking.