cEumycetoma is a debilitating, chronic, fungal infection that is endemic in India, Indonesia, and parts of Africa and South and Central America. It remains a neglected tropical disease in need of international recognition. Infections follow traumatic implantation of saprophytic fungi and frequently require radical surgery or amputation in the absence of appropriate treatment. Several fungal species can cause black-grain mycetomas, including Madurella spp. (Sordariales), Falciformispora spp., Trematosphaeria grisea, Biatriospora mackinnonii, Pseudochaetosphaeronema larense, and Medicopsis romeroi (all Pleosporales). We performed phylogenetic analyses based on five loci on 31 isolates from two international culture collections to establish the taxonomic affiliations of fungi that had been isolated from cases of black-grain mycetoma and historically classified as Madurella grisea. Although most strains were well resolved to species level and corresponded to known agents of eumycetoma, six independent isolates, which failed to produce conidia under any conditions tested, were only distantly related to existing members of the Pleosporales. Five of the six isolates shared >99% identity with each other and are described as Emarellia grisea gen. nov. and sp. nov; the sixth isolate represents a sister species in this novel genus and is described as Emarellia paragrisea. Several E. grisea isolates were present in both United Kingdom and French culture collections and had been isolated independently over 6 decades from cases of imported eumycetoma. Four of the six isolates involved patients that had originated on the Indian subcontinent. All isolates were all susceptible in vitro to the azole antifungals, but had elevated MICs with caspofungin. D escribed over 3,000 years ago in the Atharva Veda, and present in Mesoamerica during the pre-Hispanic period (1), mycetoma has preponderance for men of working age and children (2). Eumycetoma (fungal mycetoma), which is acquired after traumatic inoculation usually on exposed body sites, is characterized by a chronic progressive destruction of soft tissue and adjoining structures with associated tumefaction and purosanguineous sinuses that drain fungal grains (2-5). Without adequate treatment, the infection eventually invades the skeletal system, and clinical and mycological cure is impossible without extensive debridement/amputation (2-5). The extruded grains are either pale or dark (black), depending on the etiological agent (4, 5). Historically, the most common agents of black-grain eumycetoma were classified within the genus Madurella (Sordariales), which is characterized by organisms that produce dark gray, brown or black colonies, and dematiaceous hyphae which either remain sterile or sporulate only very reluctantly (4). The absence of conidiogenesis in isolates recovered from most black-grain mycetoma infections has historically hindered accurate species identification, and it was only recently that molecular approaches have demonstrated that Madurella mycetomatis (6) (Sord...