We report herein the case of a patient who developed fatal acute graft-versus host disease (GvHD) after liver transplantation (LT). GvHD occurred 18 days after LT and was characterized by skin epidermolysis, diarrhea and leucopenia. Skin biopsy showed epidermal dyskeratosis with epithelial necrosis, a lesion consistent with GvHD. Despite immunosuppressive therapy, the patient died within 24 days. In our observation, GvHD occurred although five HLA compatibilities were identified between the donor and the recipient, an apparently favorable and uncommon situation. This case further supports the qualification that LT may be complicated by GvHD and strongly suggests that minor rather than major histocompatibility antigens are the main target of allogenic interactions of GvHD. The involvement of chimerism in GvHD is controversial and requires further investigation.