We report on a 5-year 8-month-old boy suffering from spinal Langerhans cell histiocytosis (LCH), who had initial symptoms of back and abdominal pain, as well as tilting of the shoulder that mimicked hemivertebra of T10 with scoliosis, as revealed by radiography. The LCH-involved vertebra did not demonstrate the classic radiographic picture of vertebra plana until the vertebral body symmetrically collapsed about 6 months later, when the patient's scoliosis disappeared. The delayed diagnosis of LCH was confirmed by biopsy after another 6 months. Polyostotic lesions affecting C6, T5, T9-12, and L2 were found. This case represented an unusual presentation of LCH as an early disease entity, which resulted in a misdiagnosis of painful scoliosis. We believe we are the first to report LCH as a differential diagnosis of painful scoliosis.