An unusual clinical variant of porokeratosis (Mibelli's disease) appeared in a patient soon after birth; the lesions were widely distributed mostly on the right half of the body. Spontaneous recurrent ulcerations occurred over certain sites. The right foot was grossly deformed as a result of atrophic scarring after ulcerations. Biopsy specimens of the classical lesion showed cornoid lamella. It is suggested that the term "ulcerative systematized porokeratosis" be used to describe this clinical variant of porokeratosis.