•Primary lymphoma of the female genital tract accounts for 1% of extranodal lymphomas.•Approximately 13% are diffuse large B cell lymphoma of the uterus, most often involving the endometrial stroma or cervix.•The first double-expressor diffuse large B cell lymphoma confined to a uterine leiomyoma is described.
Keywords: Diffuse large B cell lymphoma; Double-expressor lymphoma; Uterine leiomyoma.