Langerhans cell histiocytosis frequently manifests as lesions of the skull. The disease can present with a wide spectrum of forms, from an isolated eosinophilic granuloma to multiple lesions with diffuse systemic involvement. The authors report the case of a 12-year-old boy with a 1-month history of left temporal and periorbital pain and headaches. Noncontrast computed tomography of the head was done at the time of initial presentation and was interpreted as normal. Over the next month, the patient continued to have headaches and periorbital swelling and began having bloody discharge from his nose. Magnetic resonance imaging of the brain ordered by his pediatrician showed a lesion in the left infratemporal fossa, left orbit, and sphenoid bone. The lesion was biopsied and confirmed to be Langerhans cell histiocytosis. We describe a novel multidisciplinary approach for treatment of this tumor.