Objective: Lymphoepithelial carcinoma (LEC) of the salivary glands is a very rare condition especially in the Caucasian population. We herewith contribute a case of a Caucasian woman with LEC of the parotid gland. Furthermore we present a literature review on LEC of the major salivary glands (MSG) of the past 32 years. Materials and Methods: We reported an own case and performed a review of the medical literature (1980-2012). Results: Case report: After a Follow-up (FU) of 5 years after treatment with postoperative IMRT and concomitant carboplatin, our patient has no signs of relapse. Review: We found 404 cases with LEC of the MSG published in 33 articles over the last 32 years. The mean age of the patients was 49 years (16-86); 56% were female. 95% of the cases have been treated and published in Asia (China, Taiwan). Only 17 of the 404 cases (4%) reported in 15 publications, Caucasian patients were affected. Information on treatment and outcome was not available in 75% and 72%, respectively. After a mean FU of 5.5 years (range 0.5-14) 66 of 84 (79%) patients with reported FU showed no evidence of disease. Conclusion: LEC of the salivary glands is a rare disease especially in Caucasian populations with only 4% of published LEC cases in the reviewed literature. In only 21% of the reports, clinical information on treatment and outcome was provided. Based on the limited information available from published reports, the value of radiotherapy remains unclear.