Stevens-Johnson syndrome and toxic epidermal necrolysis form a rare but severe disease spectrum characterized by widespread epidermal detachment. Gastrointestinal manifestations of the disease, however, are rarely described in the pediatric literature and have a high mortality among adults. There are limited data on the treatment of these cases, with conflicting evidence regarding the benefit of steroids, IVIG, or other immunosuppressive agents. We review previous instances of gastrointestinal involvement in children and report the case of a previously healthy 13-year-old who presented with the typical ocular and skin findings of Stevens-Johnson syndrome, subsequently developed severe life-threatening diarrhea, and was found to have severe esophagitis, duodenitis, and colitis on endoscopic evaluation. Treatment was initiated with an immediate, short course of steroids along with early introduction of an enteral diet via nasogastric tube, and resulted in full gastrointestinal recovery. This case highlights successful medical treatment of the first reported pediatric case of SJS/TEN with both upper and lower gastrointestinal tract involvement.