Lobomycosis is a chronic dermal infection that presents a wide spectrum of clinical- dermatological manifestations, mainly characterized by the development of keloid lesions as well as nodular, verrucoid and sometimes ulcerous forms. The etiological agent at an international level, according to the consensus nomenclature, has been called Loboa loboi, even though recently it has been accommodated as Lacazia loboi. The present review extensively covers the clinical-epidemiological aspects as well as the most outstanding historical aspects, including the Venezuelan experience and the presentation of two new cases, which substantiate the Amazon basin as an endemic area for the disease.