The systemic lupus erythematosus (SLE) population has a unique cancer risk profile. This article presents the most recent data on risk of cancer in lupus and discusses possible contributing factors. The risk of lymphoma is particularly increased in SLE and may be mediated by immunosuppressive medication. Lung cancer risk is also increased in SLE. There is a high rate of cervical dysplasia in women with SLE. A similar pathophysiology could be responsible for the trend seen in vulvovaginal and hepatic carcinomas. There is a decreased risk in SLE for some hormone-sensitive cancers, but the cause of this remains unclear.