Epidermodysplasia verruciformis is a rare genodermatosis characterized by inherited susceptibility to infection with certain papillomaviruses, which leads to the development of disseminated plane wart-like lesions. In some patients, lesions resembling pityriasis versicolor appear. Epidermodysplasia verruciformis has also been reported in immunosuppressed patients, most notably those with HIV infection. The affected patients are predisposed to development of skin and mucosal malignancies. We describe the rare occurrence of plasmablastic lymphoma in a patient with long lasting epidermodysplasia verruciformis and hepatitis B virus infection.