Background: Darier disease is a rare autosomal dominant disorder which typically manifests in adolescence. It is characterized by reddish brown papules or plaques in seborrheic distribution involving face, scalp, trunk and groin. Case Report: We present a rare form of this disorder with late presentation and involvement of multiple flexural areas and all major skin folds. The patient was initially treated as Hailey-Hailey disease and diagnosis could be made by biopsy and characterization of the hallmark lesions of Darier disease. Conclusion: Darier disease should be considered in the differential diagnosis of skin lesions present in flexural region.