Four cases of incontinentia pigmenti (Bloch-Sulzberger type) in a mother and three her daughter were presented and literatures were reviewed. In all three daughters vesicles appeared on toes at three weeks after birth and ehanged to pigmented patches surrounded by erythematous verrucous margin, which were then gradually spread to whole body. Pigmentation is dirty, irregular and bizarr. Severe itching sensation was complained and vesicles or bulla were formed easily due to scratching and external trauma. In mother, onset of the disease was not clear but generalized dirty pigmentation is showed. Dentition was delayed in 3rd daughter (Dental eruption was begun at 10 month old and 11 teeth was showed at now, 2 year old). Histopathological findings are compatible with incontinentia pigmenti and epidermo-dermaI separation is observed in a experimentally induced bullae, indicating a characterized epidermolysis bullosa. Strict limitation in the occurence of disease in females of one family, accompanying with epidermolysis bullosa simplex is unusual and interesting in genetical aspects and pathogenesis.