Darierdisease(DD)is a rare congenital acantholytic disorder characterised by persistent eruptions of greasy, hyperkeratotic papules in seborrhoeic areas, extremities and rarely in intertriginous areas.Nail abnormalitiesand mucous membraneinvolvement also occurs. A neuropsychiatricdisordercan be present occasionally. We are reporting a rare case of Darier disease in a male child born in a consanguineous marriage coexisting with Attention Deficit Hyperactivity Disorder (ADHD).