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BACKGROUND Epidermodysplasia verruciformis (EV) is a rare autosomal recessive genetic disorder of the immune system manifested by increased susceptibility to cutaneous human papillomavirus (HPV) infection beginning from the early years of life, and compromising lesions resembling flat warts, especially on the distal extremities and the face; but malignant transformation occurs in sun-exposed areas within the third or fourth decade of life. OBJECTIVE We describe two case reports of epidermodysplasia verruciformis, one of which was onset and had more aggressive features than the usual presentation. METHODS Intermittent surgical excision of the tumoral lesions were performed in the first case, whereas only one surgical intervestion was sufficient in the second case. RESULTS The early onset case was more aggressive, and new tumoral lesions were seen, whereas the other patients was free of the disease for 2 years.CONCLUSION Epidermodysplasia verruciformis possess multi factorial etiologies, and the main prognostic factor of the behavior of the disease may be the family history and genetic susceptibility.