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Acrokeratosis verruciformis of Hopf (AKV) is a rare genodermatosis presenting as multiple plane wart-like lesions symmetrically distributed on dorsum of hands and feet. Its pathogenesis is unknown. A case of 24 years old female is described with multiple hyperpigmented, hyperkeratotic papules on the left side of body since 5 years along the lines of Blaschko. Histopathology showed changes of AKV. Other unusual findings were the absence of family history, extensive lesions with flexural, and genital involvement.