Acrokeratosis verruciformis of Hopf is an autosomal dominant genodermatosis usually presenting with multiple planar wart-like lesions, typically observed on the dorsum of the hands and feet. The disease is very rare and the pathogenesis remains unknown. Considerable controversy surrounds the nature and relationship of acrokeratosis and Darier disease and whether they are manifestations of one genetic abnormality. We describe the case of a 19-year-old man seen in our clinic with skin-coloured, flat, warty papules localized to the dorsum of the hands and feet. Both clinical and histological findings were compatible with acrokeratosis verruciformis. We also review the disease, particularly its relation with Darier disease and therapeutical options.