Mycetoma is a chronic, suppurative and debilitating granulomatous infection seen mainly in tropical and subtropical areas and is now declared as a neglected tropical disease by the World Health Organization. The clinical diagnosis is usually characterized by a classical triad of localized swelling, underlying sinus tracts, and production of grains or granules, but unusual presentations are also seen. It is classified into eumycetoma caused by the fungus, and actinomycetoma caused by the bacteria. The clinical presentation of both is almost similar and a definite diagnosis is essential before starting the treatment as it differs for both. Surgical debulking followed by a prolonged course of medical therapy now forms the mainstay of treatment due to the long course of the disease and suboptimal response. This review focuses on the various usual as well as unusual clinical presentations of mycetoma, established treatment regimens as well as recent changes in the mode of administration of drugs and newer drugs for mycetoma.