Background: Primary bone marrow diffuse large B-cell lymphoma (PBM-DLBCL) is a rare hematological malignancy with a poor prognosis. The lack of a clear definition and unified diagnostic criteria for this disease in the WHO classification poses challenges for its diagnosis and management. Patients and Methods: To gain insights into the clinicopathological characteristics of PBM-DLBCL and explore prognostic factors, we conducted a study using data from our center and published literature spanning from 2001 to 2022. A total of 70 patients were included in our analysis. Results: Univariate analysis revealed that patients presenting with B symptoms and higher IPI scores tended to have a poorer prognosis, although statistical significance was not achieved (P>0.05). However, patients who attained a complete response (CR) after initial therapy exhibited better outcomes (P=0.007). Intensive treatment regimens demonstrated potential benefits over general treatment approaches. Additionally, our investigation suggested that rituximab-based chemotherapy was associated with improved prognoses compared to other general regimens. Conclusion: PBM-DLBCL is an aggressive disease with distinct clinical, pathological, and imaging features. It is crucial to understand the clinical characteristics of PBM-DLBCL, identify clinical risks in a timely manner, and determine appropriate treatment regimens.