Inflammatory linear verrucous epidermal nevus is a rare benign epidermal nevus that classically presents in childhood with pruritic, linear, erythematous, scaly coalescing papules and plaques distributed along the lines of Blaschko. We report a 28-year-old woman with a long-standing history of photosensitive pruritic dermatosis, exhibiting minimal improvement with topical therapies, including clobetasol, cholesterol/lovastatin cream, and ruxolitinib. Histologic evaluation revealed psoriasiform epidermal hyperplasia with focal cornoid lamella. The combination of clinical and histologic features led to the diagnosis of porokeratotic inflammatory linear verrucous epidermal nevus, a proposed variant of inflammatory linear verrucous epidermal nevus with overlapping features of linear porokeratosis. The patient demonstrated substantial improvement following CO2 laser therapy, highlighting its potential as an effective treatment option for this variant of inflammatory linear verrucous epidermal nevus.