Darier disease (DD), also known as keratosis follicularis or Darier-White disease, is a rare autosomal dominant genodermatosis that presents as hyperkeratotic, warty papules affecting the seborrheic and intertriginous areas. Patients with DD are at risk of secondary infections including the rare complication of Kaposi varicelliform eruption (KVE), a widespread viral infection most commonly caused by herpes simplex virus (HSV). Darier disease with secondary KVE can lead to widespread systemic infection and death. This case report discusses an individual with DD who subsequently developed KVE due to disseminated HSV type 2 infection.