Atypical presentation of langerhans cell histiocytosis of temporal bone in a toddler

Ever since Langerhans cell histiocytosis (LCH) was first described in 1865, mystery revolving around its cause and pathogenesis remains, although most agree that LCH is either a reactive or neoplastic process. We aim to highlight the importance of careful investigations of common presentation, which may lead to the diagnosis and treatment in a toddler. We report a case of LCH of the temporal bone with an atypical presentation in a toddler, which led to delayed diagnosis. The patient presented with a vague preauricular swelling and aural polyp. Imaging and histopathological examination of the biopsy revealed temporal bone LCH, and the child was referred to the pediatric oncology unit and successfully treated. This case clearly demonstrates the highly diversified clinical manifestation of LCH and the high level of suspicion required to diagnose it. We describe the challenge faced in managing this rare entity.