Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells. We present the case of a 9-year-old girl complaining of recent belt-like dorsalgia, whose imaging revealed dorso-lumbar scoliosis and a tumor mass at the posterior arch of T11 with posterior epidural infiltration. Surgical decompression and tumor resection were performed, histopathologically confirming the diagnosis of LCH. This abstract highlight the diagnostic and therapeutic challenges of LCH, emphasizing the importance of a multidisciplinary approach for effective management of this rare disease. Additionally, it underscores the relevance of early intervention and meticulous follow-up to ensure optimal outcomes for patients with LCH.