LCH is associated with a diverse range of neurologic symptoms, depending on the location of disease. The classic site of involvement is the hypothalamic-pituitary axis, often resulting in diabetes insipidus. Involvement of the anterior pituitary has been described. LCH can also affect the choroid plexus and meninges, giving rise to clinical features of leptomeningitis. Cerebrospinal fluid outflow obstruction can lead to increased intracranial pressure and hydrocephalus. MRI is nonspecific and can include white- and gray-matter lesions with varying enhancement, extraparenchymal involvement, and, with chronic disease, atrophy. Seizures are a clinical manifestation of LCH, classically in the setting of space-occupying lesions (not evident in this case). Untreated, LCH can cause progressive neurodegeneration, resulting in cognitive and/or motor dysfunction. Treatment of CNS disease may include surgery, radiation, chemotherapy, and/or corticosteroids.