Epidermodysplasia verruciformis (EV) is a rare, inherited dermatologic condition demonstrating an increased susceptibility to specific HPV genotypes, resulting in both benign and malignant skin lesions. Epidermodysplasia verruciformis lesions are frequently described as pityriasis versicolor-like scaly macules, flat wart-like papules, or verrucous and seborrhic keratosis-like papules and plaques. Acquired EV has been described in patients with HIV and in those who are on immunosuppressive therapy. We discuss a patient with congenital EV who presents with skin lesions associated with HPV 10, a less frequently cited causative subtype, and histological findings that are not classic for EV.