Eosinophilic granuloma (EG) is a benign and solitary bony lesion of unknown etiology, which typically affects children: 90% of patients are between the ages of 5 and 15 years. EG, a variant of histiocytosis X, is a rare disease causing destructive bony lesions of the cervical spine in children. This report describes the case of a 5-year-old boy with torticollis who presented to the Emergency Department and was found to have EG of C6. He was successfully treated with anterior cervical corpectomy and fusion by using anterior plate and screws, with resolution of his symptoms.