Since birth, a 24-year-old female Hispanic homemaker, a resident of Valença, State of Rio de Janeiro, Brazil, had warty lesions between the second and third fingers of the left hand, as well as on the left side of the vulva. She had been referred to the Sexually Transmitted Disease Clinic for further investigation. Previous histopathological biopsy findings of the lesions indicated the possibility of epidermodysplasia verruciformis, but polymerase chain reaction (PCR) did not confirm the presence of human papillomavirus (HPV) DNA. At the age of 15, she had undergone surgery for excision of some lesions. Five years after her two term pregnancies, lesions began to develop mainly in the vulva and thigh, for which she did not undergo any cosmetic surgery. Those lesions continued to develop during the third pregnancy. Her 2-year-old daughter also showed verrucous lesions in the right axilla and finger nail bed on the left hand, indicating the possibility of a genetic disorder. Clinical examination of the patient’s lesions showed well-demarcated vulvar verrucous lesions, linear lesions on the left hand, and dark brown papules having linear distribution, principally on the left side of the body (Figures A and B) and left inframammary region and the sternum. A previous cesarean section scar also had verrucous lesions. A histopathological exam of a new biopsy specimen confirmed the diagnosis of inflammatory linear verrucous epidermal nevus (ILVEN), which remains of an unknown etiology (Figure C). PCR performed again for HPV indicated negative results. The patient was treated with 90% trichloroacetic acid every week for 4 weeks, with which the lesions resolved. The patient is currently being followed up clinically in a gynecology assistance program.