Background Previous studies of mortality associated with GCA have shown conflicting results. We conducted a systematic review and meta-analysis to determine the mortality risk in GCA patients compared to the general population. Methods We searched for published studies indexed in MEDLINE and EMBASE and the Cochrane database from inception to June 18, 2015 using the terms “giant cell arteritis” and “temporal arteritis” combined with the terms for death, mortality, and survival. A manual search of citations from retrieved articles was also performed. The inclusion criteria were as follows: (1) observational studies of mortality in GCA and (2) comparison of mortality to the general population. Studies published only in abstract form were excluded. Study eligibility and quality (Newcastle–Ottawa scale) were independently assessed by at least two investigators. Random effects meta-analysis of the mortality ratio (MR) was performed by the inverse variance method. Results Out of 435 potentially relevant articles, 64 studies were reviewed, 19 studies were included in the review and 17 studies were included in the meta-analysis. Mortality was not increased in GCA patients ascertained from a population base (MR = 1.03, 95% CI: 0.96–1.10), but was increased in patients ascertained from a hospital setting (MR = 1.61, 95% CI: 1.19–2.19). There was no difference in MR by gender, and two studies provided evidence that mortality was increased in the early years following diagnosis. Conclusion At a population level, long-term mortality is not increased in GCA. However, mortality risk may be increased in some patients, and may vary over time.