Introduction: Familial Mediterranean fever (FMF) is an autosomal recessive disease considered to be the most common entity of a rare group of disorders known as auto-inflammatory syndromes which have acute presentations in emergency settings. Methods: A search of Web of Science, Google Scholar, Cochrane, and PubMed databases for articles published before January 2019 was performed using relevant keywords. Full-text English articles, including review articles, case series, and experimental studies, were evaluated. Unrelated studies were excluded. Based on the selected keywords, 440 studies were selected for review. Ultimately, 60 met the study criteria for final assessment. Results: Given the various symptoms and the multiplicity of differential diagnoses, physicians may easily miss diagnosing FMF. Accordingly, emergency medical staff must be trained in order to significantly reduce the number of medical errors and economic costs and to improve the quality of life of involved patients. Conclusion: Although FMF is already known to be an inflammatory entity, more study and investigation of it is required. There is an educational gap in both medical and general populations that should be filled by using new genetic testing and providing appropriate social and medical education.