Langerhans cell histiocytosis (LCH) is a rare condition of unknown etiology characterized by infiltration of one or more organs by Langerhans-type dendritic cells, most often organized into granulomas. This entity was initially described in young adult children. We report an infant case of 4 years old boy with clinical particularities such as tinnitus, imbalance of movement coordination, and itching. The MRI revealed a temporal mass lesion. Surgical management was performed by direct excision. The tumor excision was subtotal with simple postoperative outcomes, and good clinical improvement at discharge. Then we address him to the pediatric oncology hospital center. We will present also the state of current knowledge concerning infant LCH and recent data to guide therapeutic management. LCH has an extremely polymorphic clinical presentation. Its management in children must be multidisciplinary between neurooncologists and pediatric neurosurgeons.