Darier's disease is a rare genetic disorder with autosomal dominant inheritance. It is characterized by hyperkeratotic papules in seborrheic areas. Associated abnormalities include nail abnormalities and changes in the mucous membranes. Exacerbation of the disease occurs with exposure to high temperatures, sun, and sweating, resulting in a worsening clinical picture in summer months. The unilateral zosteriform pattern is a rare variant that is clinically manifested by a unilateral outbreak of erythematous keratotic papules without any other associated symptoms. Here we present a 52-year-old male with a zosteriform pattern of Darier's disease. We also discuss the most important clinical and pathohistological characteristics of the disease and various treatment options.