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Abstract Background Primary hepatic lymphoma is a very uncommon disease. Due to its nonspecific clinical, laboratory, and imaging findings, it is often misdiagnosed. Liver biopsy is required to make a final diagnosis. Chemotherapy is the current gold standard of treatment. Case presentation An asymptomatic 65-year-old Caucasian man with Child-Pugh class A cirrhosis presented to our hospital with a nodular lesion seen on a routine surveillance abdominal ultrasound. His physical examination revealed hepatomegaly and no other significant findings. Magnetic resonance imaging of the abdomen showed a voluminous nodule on the left lobe with heterogeneous contrast enhancement. His liver biopsy was compatible with diffuse large B-cell lymphoma. Systemic staging showed no evidence of nodal or bone marrow involvement, confirming the diagnosis of primary hepatic lymphoma. He was treated with chemotherapy. However, he developed febrile neutropenia after one of the cycles and died. Conclusions In this article, we report a rare presentation of non-Hodgkin lymphoma and review the current literature on clinical features, diagnosis, and management.