Lobomycosis is a cutaneous infection of tropical and subtropical regions caused by the fungus Lacazia loboi, which still has a controversial taxonomical position. The first description of the disease and fungus was made in 1930 by Jorge Lobo. It is a chronic disease with predominance of lesions similar to keloids, in exposed areas, limited to skin and semimucosa. There is no systemic involvement and patients maintain a good general health. Diagnosis is confirmed by direct or histopathologic exam and, until present, the fungus has not been cultivated. Surgery is the treatment of choice for isolated lesions, but there are frequent recurrences. Good therapeutic responses have been reported with clofazimine, with or without itraconazole, and with 5-fluorocytosine. This disease should be recognized by dermatologists worldwide because, although restricted to the Amazon region, it has been observed in other locations. Research development and achievement of new knowledge in molecular biology and genetic engineering of lobomycosis are of utmost importance because they may, in the future, lead to the culture of the fungus in the laboratory and to a better understanding of its pathogenesis, transmission mechanism, and new methods of diagnosis, prevention and treatment.