Abstract: Primary bone lymphoma (PBL) is a rare form of lymphoma originating within the skeletal system, which may present as either solitary or multiple osseous lesions. At diagnosis, there is no involvement of distant or widespread lymph nodes, nor evidence of extraosseous disease in other organs. Establishing a diagnosis requires exclusion of systemic lymphoma with secondary bone involvement and typically involves a comprehensive assessment of imaging, histopathology, and clinical staging. PBL accounts for approximately 1%–2% of non-Hodgkin lymphoma (NHL). Overall, the prognosis of PBL is relatively favorable, with reported 5-year overall survival (OS) rates ranging from 58% to 88%. However, due to its often insidious clinical presentation, misdiagnosis is common, and definitive diagnosis relies on a combination of biopsy and imaging studies. In recent years, advances in molecular classification and targeted therapies have significantly improved patient outcomes, although the role of radiotherapy and the necessity of central nervous system prophylaxis remain subjects of ongoing debate. This research reviews the latest domestic and international evidence and presents an expert consensus on the diagnosis and management of PBL.