Leila Ghahramani - Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Ahmad Izadpanah - Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Ali Mohammad Bananzadeh - Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Majid Akrami - Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Faranak Bahrami - Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Seyed Vahid Hosseini - Colorectal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

Neurofibromatosis type ۱, also known as von Recklinghausen disease, is an autosomal dominant disorder with an incidence of ۱ per ۴۰۰۰. Neurofibromas are benign, heterogeneous tumors arising from the connective tissue of peripheral nerve sheaths, especially the endoneurium. Visceral involvement in disseminated neurofibromatosis is rare. Neurofibroma occurs most frequently in the stomach and jejunum, but the colon and anorectal canal may also be involved. Gastrointestinal neurofibromas may lead to bleeding, obstruction, intussusception, protein-losing enteropathy and bowel perforation. We present a case of diffuse involvement of the anorectal area by a huge neurofibroma, with pelvic pain, watery diarrhea and urgency.