Plasma cell vulvitis (PCV) is a rare disease affecting anogenital skin. It is also known by multiple names including Zoon’s vulvitis and vulvitis circumscripta plasmacellularis. It is an inflammatory condition that can cause severe local discomfort and psychosocial distress. The diagnosis is challenging and often remains unrecognized. It usually presents as a glistening, red, well-demarcated plaque or patch. The pathophysiology is unknown. Treatment recommendation is mostly based on case reports and case series and this condition is often recalcitrant to topical and systemic treatment.