Zygomycosis (mucormycosis) is a rare fungal infectious disease, usually found in association with an immunocompromised state. Gastrointestinal mucormycosis is extremely rare and fatal, thus it is important to detect and manage this disease at an early stage in an effort to improve survival. To date, no cases of mucormycosis superimposed on gastrointestinal Behcet’s disease have been reported. Herein we report a case in which gastrointestinal mucormycosis occurred in a 17-year-old-female with Behcet’s disease. The patient recovered from her disease after undergoing an ileocecectomy