Conclusions: Systemic lupus erythematosus (SLE) is associated with an increased risk of developing a head and neck malignancy (HNM). A history of SLE did not significantly impact the survival of our study cohort after cancer developed. Objectives: To examine the risk and survival rates of HNM in patients with SLE. Methods: This was a population-based, retrospective cohort study. We compared patients newly diagnosed with SLE between 2001 and 2008 (n = 8751) with age-matched controls (1: 10) (n = 87 510). The incidence of HNMs at the end of 2009 was then determined. Results: We found a 2.16-fold higher risk of HNMs in patients diagnosed with SLE compared with the risk of first malignancy in the age-matched controls (incidence rate ratio, IRR = 2.16, p < 0.05). The site with the highest incidence of HNMs in SLE patients was the oral cavity (5/11, 45.45%), followed by the nasopharynx (4/11, 36.36%). SLE displayed no synergic effect on the survival of SLE patients with an HNM compared with age-matched controls with a new HNM (p = 0.2446).