In a retrospective analysis of 108 patients with lymphoepithelioid cell lymphoma (LeL) (median age 60, range 21–87 years) initial extent of disease was Ann Arbor stage I in 9%, II in 19%, III in 35%, and IV in 37% of the patients with a high incidence of generalized lymphadenopathy (71%) but seldom extralymphatic disease (13%). Unstable spontaneous remissions were observed occasionally. Complete response rates were 75% in the patients treated with radio‐ and/or chemotherapy but relapse occurred in 65%. Median overall survival was 16 months with a significantly better prognosis for stage I/II than for stage III/IV disease (P= 0·014). LeL thus seems to possess an unfavourable prognosis possibly to be improved by standardized treatment approaches.