Darier’s (Darier–White) disease/keratosis follicularis was initially reported in the year 1889 by Darier and White independently. Darier’s disease has since (for over a century) fascinated research workers. 1–12 Almost all cases of the disease are attributed to mutations in the sarcoplasmic endoreticulum Ca 2+ -ATPase isoform-2 (SERCA-2). 13–24 The original suggestion that dyskeratotic cells represented an organism was dispelled long ago. 25 The genetic nature of the disease was initially suggested for the first time by White, when he reported the simultaneous affliction of a mother and her child. 25 The discovery that the disease is due to specific mutations has provided an excellent opportunity to study its genetic epidemiology. 22–24 The present update is an attempt to comprehensively review the developments in the field to date, and to correlate the various facets of the entity.