Objective To discuss the clinical features, diagnosis and therapy of primary adrenal lymphoma (PAL) . Methods Two patients (65-year old and 52-year old respectively) who complained vague pain were analyzed. Occupying lesions in adrenal gland were discovered by computed tomography (CT) and diagnosed by needle biopsy and laparoscopic surgery. Results The two patients in our hospital were diagnosed as nonHodgkin's lymphoma. They were treated with chemotherapy and one of them was partial response. Literatures on PAL were reviewed. In the literature, 7 of the 65 patients achieved complete response, 15 partial response, 43 death or unspecified and the survival duration was 3 days to 26 months. Conclusions Most of PAL are bilateral, without specific clinical characters. Neele biopsy is the first choice to make the diagnosis. The therapeutic modalities for PAL include surgery, chemotherapy, surgery followed by chemotherapy as well as radiation therapy. The prognosis of PAL is poor if other organs are involved.