As Allium cepa is one of the most important condiment plants grown and consumed all over the world, various therapeutic and pharmacological effects of A. cepa were reviewed. Onion (Allium cepa) is a high dietary fiber-rich perennial herb that is placed under the family Amaryllidaceae. It contains high concentration of folic acid, vitamin B6, magnesium, calcium, potassium, and phosphorus as well as vitamins and minerals. It is widely used as an antimicrobial agent, but it showed anticancer, antidiabetic, antioxidant, antiplatelet, antihypertensive, and antidepressant effects and neuroprotective, anti-inflammatory, and antiparasitic effects and so on. It is said to have beneficial effects on the digestive, circulatory, and respiratory systems, as well as on the immune system. This review article was devoted to discussing many health benefits and traditional uses of onions in pharmacological perspectives, as well as the safety/toxicological profile. If more detailed research on this perennial herb is conducted, it will open the door to an infinite number of possibilities.