Dominant dystrophic epidermolysis bullosa-pretibial (DDEB-Pt) is an extremely rare subtype of dominant dystrophic epidermolysis bullosa (DDEB). Clinically, DDEB-Pt is characterized by trauma-induced blistering with scarring that predominantly affects the pretibial region and causes nail dystrophy. A 42-year-old woman had recurrent numerous pruritic lichenoid papules and plaques and a few vesicles on both the pretibial areas with toenail dystrophy for over 30 years. Her son and daughter also had the same lesions on their pretibial areas with associated dystrophic toe nails. Herein we report on a case of DDEB-Pt occurring in one family as a rare case. (Korean J Dermatol 2010;48(7):606∼610)