Lymphoid neoplasm with 18q21.3/BCL2 and 8q24/MYC translocation to immunoglobulin genes as dual-hit lymphoma in female bilateral ovaries is rare and has a poor clinical outcome. The present study reported on the case of a 33-year-old female, who was admitted to the hospital due to lower abdominal distension aggravated during defecation but with no obvious inducement. The B-ultrasound revealed bilateral adnexal solid masses and the pathological examination indicated advanced B-cell lymphoma (HGBL) with MYC and BCL2 gene rearrangement. The patient then received R-CHOP treatment, but the effects were poor. Rare extranodal HGBL presentations with MYC and BCL2 rearrangement should be considered in the differential diagnosis of masses at unusual sites, such as the adnexa. Due to their aggressive nature, early and prompt recognition of these lymphomas is essential for appropriately administering therapies.