Intra-osseous schwannoma is a rare mesenchymal tumor. Although, the head and neck region is one of the most common sites for schwannomas, its location at the skull bone is uncommon and accounted for less than 0.2% in the largest series of bone tumors ever reported. Furthermore, it is most often a benign tumor, malignant transformation is exceedingly rare.Clinical presentation is non-specific, most often symptoms are associated with compression and invasion of adjacent organs. Neuro-imaging features are non-specific and the diagnosis is based on histological examination with immunohistochemical study.Surgery remains the aim of treatment. However, radiation therapy could be an interesting therapeutic option in unresectable tumors.This systemic review offers new clinicopathological data useful for better defining the diagnosis and clinicopathological behavior of schwannoma. The purpose of this work is to raise awareness among clinicians adding this clinical entity as a differential diagnosis when a mass of skull bone is identified.