Calvarial lymphoma is an exceedingly rare phenomenon; the clinical presentation and imaging pattern mimic many diseases of the central nervous system. Several treatment approaches have been undertaken with variable use of surgery plus adjuvant chemotherapy and radiation; an optimal treatment algorithm has yet to be defined. The aim of this study was to better characterize management strategies and patient outcomes. An illustrative case was presented and a meta-analysis was carried out in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. MEDLINE and Embase were searched for cases of calvarial lymphoma. Outcomes between patients who underwent open surgery and biopsy only were compared directly. In an analysis from 1976 to 2019, 62 patients with a median age of 60 were included. The most common presentations were subcutaneous scalp mass (89%), headaches (26%), and focal neurological deficits (21%). Osteolytic changes on computed tomography were seen in69% of patients with extension into either the intracranial or extracranial space in 97% of cases. Surgical excision was performed in 41 patients with a remission rate of 85%and a recurrence rate of 5%, which didnotvary significantly from patients treated nonsurgically (remission in 75%, P= 0.479; recurrence in 0%, P= 1.000) CONCLUSIONS: In patients presenting with a progressively enlarging scalp mass, calvarial lymphoma should be in the differential diagnosis, as it can be effectively managed with a biopsy followed by chemotherapy and radiation. The role for open surgery may be limited.