BackgroundAnal lymphomas are extremely rare. There are no relevant descriptions in professional books, and there are only a few case reports in the literature. Here, we report a new case and review the literature to summarize the clinical and pathological features of anal lymphoma.MethodsWe described a case of anal lymphoma confirmed by pathological diagnosis, then searched the PubMed database, and finally selected 12 reported cases to be included in the study. We described the clinical and pathological characteristics of the patients.ResultsThirteen patients with anal lymphoma were confirmed. Seven men and six women with a median age of 50. There were four cases of HIV- and EBV-infected patients. The size of the tumor was 1–13 cm, all of which were diagnosed as B-cell lymphoma, and 61.5% were diffuse large B-cell lymphomas. Among the 13 patients, eight received chemotherapy or immunochemotherapy, two received radiotherapy, one received chemotherapy combined with radiotherapy, one received surgery, and one gave up treatment. Three patients died, and only 2 of 10 surviving patients had complete remission.ConclusionAnal lymphoma is extremely rare. Patients with persistent abscess complicated with HIV or EBV infection should undergo pathological biopsy to exclude anal lymphoma.