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Background and importance: Scheuermann’s disease (SD), intradural-extramedullary epidermoid cysts and idiopathic scoliosis (IS) are all rare conditions. The co-occurrence of the three condition on one case has not been reported in literature before. We describe an extremely rare case of SD co-occurrence with IS and intradural-extramedullary epidermoid cyst, which is the first in the literature.Clinical presentation: A 13-year old boy and his parents presented at Honghui Hospital complaining of progressive hyperkyphosis for 5 years and back pain for 2 months. Thorough medical examination and radiology confirmed the diagnosis of SD kyphosis, IS, and intradural-extramedullary epidermoid cyst. Medical panel with spine surgeons, oncologists, neurosurgeons, histopathologists and radiologists were consulted. Tumor excise, kyphosis recorrection and spine reconstruction surgery was conducted. Postoperative histological examination detected desquamation of keratin from the epithelial lining and stratified squamous epithelium without skin adnexa. The boy was ambulant 2 days postoperative with walking aids.Conclusion: This is the first case report of Scheuermann’s disease (SD) co-occurrence with idiopathic scoliosis (IS) and intradural-extramedullary epidermoid cyst. The co-occurrence of intradural epidermoid cyst, SD and IS might not be a coincidence, indicating a kind of genetic etiology need to be illuminated.

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A Case of Scheuermann’s Disease Co-Occurrence with Idiopathic Scoliosis and Intradural-Extramedullary Epidermoid Cyst: The First Case Report in Literature