Lymphocytic infundibulo-neurohypophysitis (LINH), a rare autoimmune disease, is distinct from lymphocytic hypophysitis and is characterized by lymphocytic and plasma cell infiltration of the posterior lobe of the pituitary and the pituitary stalk.(1) We report the case of a young boy who presented with recurrent, steroid-responsive optic nerve dysfunction and diabetes insipidus. T1-weighted magnetic resonance imaging scans of the brain showed an isointense soft tissue mass within the sella turcica and a thickened pituitary stalk. Tissue specimen resected at transsphenoidal surgery revealed chronic inflammation with infiltration of lymphocytes and no granulomatosis or necrosis, establishing the diagnosis of LINH.(2) This is the first report of optic neuropathy in association with LINH in a child.