The eumycetoma is a severely debilitating chronic progressive fungal cutaneous infection. Classic clinical triad is characterized by painless subcutaneous mass, sinus tracts formation and sero-purulent discharge that contain aggregates of fungal hyphae called grains. Any part of the body can have affected, with extension to muscular or bone, even visceral compromised. The eumycetoma is observed in tropical and subtropical countries; In Latin-America, is reported with less frequency. In endemic areas, antibody presence again etiological agents were higher compared with number of people affected, thus it is supposed that individual genetic susceptibility most by exist. Recently, it was reported specific polymorphism in genes CR1, IL-8, NOS2 and chitriosidase, which were associated with development of eumycetoma. The diagnosis is suggested by clinical presentation; the histopathology and microbiology studies, plus radiologic valuation confirmed diagnosis. Madurella mycetomatis is the most informed etiological agent. Using phylogenetic tools new species in genus Madurella were reported; moreover, Trematosphaeria grisea and Pseudallescheria boydii were reclassified. Etiological agent Identification is important, because differences in antifungal susceptibility exist. Eumycetoma treatment includes surgery plus antifungal drugs. Identification of etiological agents is primordial, because antifungal resistance could exist. To development new pharmacological strategies, comprehension of grain formation physiology and drugs effects are necessary.