A true primary malignant lymphoma of the bone is definedas a solitary mass lesion with no evidence of disease at others sites and no systemic dissemination within 6 months of detection of tumor. [1,2] Solitary, diffuse, and multifocal skull lesions fulfilling other criteria of primary malignant lymphoma were kept as selection criteria for our study. The electronic databases (Pubmed and Google Scholar) were searched using key words like extranodal lymphoma, non-Hodgkin lymphoma (NHL) and primary skull lymphoma. Titles and abstracts of the initially identified studies were screened to determine if they satisfied the selection criteria. Full-text articles were retrieved for the selected titles. Reference lists of the retrieved articles were searched for additional publications. We found that sporadic cases have been reported in different journals. [1-23] The patients have variable clinical presentation and management. To our knowledge, this is the largest series of 25 reviewed cases of primary skull lymphoma ever reported.