A case is reported of a 20 year-old man who had progressive poikiloderma, cutaneous atrophy, plantar keratosis, and, from birth, blister formation regressive in infancy. Ultrastructural study performed on poikilodermic and atrophic lesions showed that dermoepidermal "clefts" or " vacuolizations " observed in optical microscopy did correspond to a cleavage between lamina densa and undamaged basal cells membrane. Sixty cases of congenital poikiloderma with blisters and keratosis have been reviewed in the literature; 13 out of these, are as the present case, similar to the original observation of Th. Kindler ; the common clinical features of these cases (cutaneous atrophy, mucous membrane involvement) and probably autosomic recessive inheritance, allow to consider, according to the authors opinion, Kindler 's syndrome as a peculiar entity.