Primary esophageal diffuse large B-cell lymphoma (DLBCL) is an extremely rare clinicopathologic entity. We report one case from our clinical experience and undertake a review of the previously published cases. A systematic literature search of the medical databases PubMed and Google Scholar was conducted. A total of 15 cases fulfilled the inclusion criteria. The data on patients' characteristics, epidemiology, clinical features, HIV status, gross appearance of the lesion, esophageal location, treatment, and outcome were collected and analyzed. Primary esophageal DLBCL was more common among males, primarily in the fifth and sixth decades of life. Dysphagia was the most common initial clinical presentation. Tissue biopsy with immunohistochemistry was an indispensable diagnostic modality. The mainstay of treatment was chemotherapy with cyclophosphamide, vincristine, doxorubicin, and prednisone regimen, in addition to anti-CD20 antibody rituximab, with or without radiotherapy. This review serves to outline our current understanding of the epidemiology of and risk factors for primary esophageal DLBCL, the pathophysiology of this disorder, and currently available approaches to diagnosis and management.