The authors reported a case which does not seem to be possibly related to any known clinical entity; an aspect of keratosis lichenoides striata is associated with an ichthyosiform condition of the trunk and a pseudo-ainhum; there is also a neurological syndrome similar to a multilocular sclerosis. This patient resembles the previous cases of the literature; however there is neither erythema nor any striation at the back of the knees. There is also a pseudo-ainhumm. Concerning this observation, a review of the literature on keratosis lichenoides striata is presented.