Introduction: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder and its incidence is not exactly known. It occurs in all age groups but is more common in the pediatric population. It is characterized by single or multiple osteolytic-type lesions caused by clonal proliferation of cells histologically like Langerhans cells; its clinical presentation is heterogeneous.

Clinical case: An 11-year-old man with a 6-month history of back pain and walking limitation. Magnetic Resonance Imaging (MRI) showed a lesion of the dorsal spine in D8, D9, and D10 and flat vertebra D9 that caused spinal compression. The diagnosis was made based on the histopathological study of the vertebral body with the finding of eosinophilic granuloma, being treated with outpatient chemotherapy, external fixation with a plaster corset, and physical therapy. The clinical evolution was favorable, achieving improvement in muscle strength and walking with support at discharge.

Conclusion: Langerhans cell histiocytosis with vertebral involvement is a highly relevant pathology, despite being rare. Timely diagnosis and adequate treatment are essential since it allows to prevent or limit the spinal cord involvement caused by this pathology.