Darier-White's disease, keratosis follicularis, is a rare keratinizing skin disorder that occasionally manifests mucosal lesions. The oral mucosa is more commonly involved than that of the uvulva, pharynx and larynx. On the oral mucosa, superficial erosion, minute white papules and hyperkeratotic plaques are evident. The cause is unknown. It is progressive in severity with age and is inherited as an autosomal dominant trait affection both sex equally. The most characteristic or diagnostic features are the peculiar benign dyskeratosis and the suprabasilar lacunar formation, which are characterized by the presence of unusual cells, corps ronds and grains. Therapy with derivatives of vitamin A, the retinoids, is known to be most effective. Recently the authors have experienced a case of Darier-White's disease in 28-year-old woman involved oral and pharyngeal mucosa.