Primary hepatic lymphoma (PHL) is a very rare malignancy and is characterized by liver involvement at presentation with no affectation of the spleen, lymph nodes, peripheral blood, bone marrow, or other tissues until at least 6 months after diagnosis. PHL should be considered in the differential diagnosis in a patient with space-occupying liver lesions and normal levels of alpha-fetoprotein and CEA. A computed tomography (CT) scan is the commonly used modality for staging lymphomas. The widespread use of positron emission tomography/ CT results in the improvement in the accuracy of detecting the extent of disease, response evaluation, and prognostication. The liver biopsy, due to its pleomorphic appearances in the needle biopsy specimen, can be very challenging. Current literature favors the combination of chemotherapy as the frontline treatment for its least invasiveness and improved survival. Favorable prognosis of PHL can be obtained by early surgery combined with chemotherapy in strictly selected patients. However, the optimal therapy is still unclear and the outcomes are uncertain.