A 35-year-old man sustained Hallopeau-Siemens recessive dystrophic epidermolysis bullosa (RDEB-HS) confirmed by gene analysis. A detailed study of mutation analysis was carried out by Dr. Gau-Tyan Lin[]. A homozygous intronic splice-site mutation at the +1 position of intron 5 (682+1G→A) in the type VII collagen gene (COL7A1) was identified. His mother was a heterozygous carrier of this mutation[]. Parental consanguinity was confirmed by the DNA study. With early onset at 7th day postnatal, he suffered blisters and erosions at all skin of his body. He had a characteristic mitten deformity of hands and feet. Since the age of 21 years, he had received lots of surgical procedures including separation of digits, release of flexion contracture, and skin grafting for ulcers, skin defect and pseudosyndactyly of his hands and feet. In the meanwhile, repeated skin infection was experienced. Besides he had anemia, malnutrition, and chronic constipation with stool impaction for years. Laxatives (magnesium oxide 250 mg 3 times per day and sennosides 20 mg before sleep) were given. Due to skin ulcers and wound infection of right hand and right low leg, he was admitted to the hospital. He received wound debridement of right low leg and right hand with separation of first web of right hand. Three days after the operation, abdominal pain and distention were complained. Enema (glycerol 60 ml) was given, but to no avail. Vomiting and abdominal discomfort got worse. White blood count: 25.50 × 1000/ul was noted. Computed tomography (CT) of abdomen demonstrated extra-luminal free air in the peritoneal cavity and stool impaction in the distal sigmoid colon with marked distention of colonic loop. Hallow organ perforation with peritonitis was highly suspected. Emergent laparotomy was performed. During operation, sigmoid colon perforation with ischemic bowel and fecal impaction in the distal sigmoid colon were noted. Hartmann's procedure (resection of ischemic bowel and colostomy ) and intra-abdominal abscess drainage were done. Unfortunately septic shock developed. Intensive management was applied; however, the condition got worse, and he was expired two days after operation.