Ethyl carbamate (EC), a chemical substance widely present in fermented food products and alcoholic beverages, has been classified as a Group 2A carcinogen by the International Agency for Research on Cancer (IARC). New evidence indicates that long-term exposure to EC may cause neurological disorders. Formation of EC in food and its metabolism have therefore been studied extensively and analytical methods for EC in various food matrices have been established. Due to the potential threat of EC to human health, mitigation strategies for EC in food products by physical, chemical, enzymatic, and genetic engineering methods have been developed. Natural products are suggested to provide protection against EC-induced toxicity through the modulation of oxidative stress. This review summarizes knowledge on the formation and metabolism of EC, detection of EC in food products, toxic effects of EC on various organs, and mitigation strategies including prevention of EC-induced tumorigenesis and genotoxicity by natural products.