Abstract
Langerhans cell histiocytosis (LCH) is a disease characterized by localized and generalized proliferation of the histiocytes. It is a locally aggressive condition. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. It can present as a unifocal or multifocal disease. The majority are present in the head and neck region, but the involvement of Paranasal sinuses is rare. Here we describe a 64-years-old female who presented with a slow-growing left nasal mass for 1 year. Evaluation of the patient was suggestive of malignancy, but the biopsy report turned out to be Langerhans cell histiocytosis; subsequently left, total maxillectomy was done. We hereby present a unique case of LCH with isolated nose and paranasal sinus involvement.