Eosinophilic granuloma of the skull requires first consideration of surgical treatment. It is a benign lesion of the skull and is not a malignant tumor. However, it will grow and continuously invade the patient’s normal skull, causing an increasing extent of cranial defects, which is usually diagnosed clearly only with a cranial CT. Patients usually feel a mass on the scalp, and when the neurosurgeon conducts a physical examination, the corresponding cranial defect will be found, and the cranial defect is elevated and has some toughness. In this case, surgical excision should be actively considered for treatment. During surgery, not only the eosinophilic granuloma is removed, but also the corresponding normal cranial rim is removed. After all lesions have been removed, cranial repair surgery is considered at the same time, usually with priority given to titanium mesh cranial repair.