Introduction and importanceLangerhans cell histiocytosis (LCH) is a rare idiopathic disease that uncommonly affect the spine in adults.Case presentationIn this report, we presented a rare adult case of symptomatic spinal LCH with asymptomatic systemic involvement. She was a 46-year-old previously healthy lady who presented with subacute thoracic sensory level, urine retention, constipation, and pyramidal paraplegia. Her magnetic resonance imaging (MRI) of the spine revealed T6 compression fracture with an epidural mass compressing the cord.Clinical discussionSellar MRI showed pituitary gland enlargement with hyperintense signal in the posterior lobe. Positron emission tomography (PET)/computed tomography (CT) scan showed an increased uptake in the right parotid gland uptake and renal cortex, indicating systemic involvement.ConclusionSurgical excision, decompression, and screw fixation were performed, and the patient improved. The prognosis is usually good in patients with solitary spinal LCH.