Introduction: Epidermodysplasia Verruciformis (EV) is a rare inherited genodermatosis characterized by an abnormal susceptibility to specific types of human papillomavirus infection. The disease manifests as persistent hypopigmented macules, flat wart-like papules, and plaques predominantly over sun-exposed areas, and carries a risk of malignant transformation.

Case Presentation: We report the case of a 21-year-old female presenting with asymptomatic hypopigmented lesions over the face, neck, chest, and upper arms for six years. Cutaneous examination revealed multiple hypopigmented macules and flat wart-like papules predominantly over sun-exposed areas, along with oral hyperkeratosis. Histopathological examination demonstrated epidermal thickening with enlarged keratinocytes exhibiting prominent perinuclear halos and grayish-blue cytoplasm, consistent with EV. The patient was advised to undergo strict photoprotection and oral isotretinoin therapy was initiated.

Conclusion: This case highlights the importance of the early recognition of EV and emphasizes the need for long-term follow-up owing to the potential risk of malignant transformation.