Background: Epidermodysplasia verruciformis is a rare, inheritable disease characterized by an unusual susceptibility to infection with specific types of human papillomavirus and a greater propensity for developing malignant skin tumors. Case presentation: A 55-year old male was presented with complaint of painless, hard growth extending from his toe nail for the last one year. He was diagnosed as a case of Epidermodysplasia Verruciformis followed by Mohs surgical procedure. Conclusion: Epidermodysplasia Verruciformis is an autosomal recessive genetic disorder and it predisposes patients to widespread papilloma viral infection. This might be considered as a model of cutaneous human papilloma virus oncogenesis. Generally, Epidermodysplasia Verruciformis reveals an autosomal receding pattern of inheritance. Prolonged exposure to sunlight, living in high altitudes and outdoor occupations are the known risk factors to malignant deterioration of EV lesions.

Keywords: Case report, squamous cell carcinoma, oncogenesis, Epidermodysplaia Verruciformis, malignant growth