Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells with uncertain etiology, wide spectrum of clinical symptoms and varied behavior. Treatment is not well defined and is highly individualized. The difficulty of the therapy also lies in the unpredictability of the clinical course. Further clinical studies are required to establish the natural course and prognostic factors of" this disease and more research needs to be conducted to understand the pathogenesis and rational management of this disorder. The authors present a review of vulvar localization of LCH, and the current concept of Langerhans cell biofunction is also outlined

        Key words: Langerhans cell histiocytosis, vulva, immunohistochemistry, treatment