Inflammatory linear verrucous epidermal nevus is a rare cutaneous disorder characterized by pruritic, erythematous, and verrucous papules and plaques along the lines of Blaschko. Histopathologically, there is a benign verrucous proliferation of keratinocytes together with alternating parakeratosis and orthokeratosis as well as inflammatory changes. We report a patient who developed squamous cell carcinoma (SCC) on an inflammatory linear verrucous epidermal nevus and we discuss the importance of regular follow-up of patients with epidermal nevi.