Hailey-Hailey disease, also known as benign familial pemphigus and first described by the brothers Howard and Hugh Hailey in 1939,1 is an autosomal dominant rare genodermatosis caused by a mutation of the ATP2C1 gene that results in a defective intracellular calcium pumping mechanism.2,3 The disease is characterized by junctional abnormalities of epidermal keratinocytes and clinically presents as chronic recurrent erythematous plaques, bullae, and erosions, principally on the intertriginous regions.4,5 Involvement of the eye in Hailey-Hailey disease is uncommon. This evidence concerns the gene ATP2C1 and Hailey-Hailey disease.