Sporotrichosis is a sporadic and rare mycotic infection in most of the developed world. In many parts of the developing world, sporotrichosis is much more commonly recognized, but epidemiological data are generally lacking from these regions. We report epidemiological, clinical, and treatment data from 238 cases of culture-proven sporotrichosis occurring in a relatively remote area of the south central highlands of Peru that were retrospectively collected during 1995-1997. Most cases (60%) occurred in children aged ≤14 years, and the most commonly affected anatomic site was the face. Disease was clinically confined to the skin and subcutaneous tissue in all patients. The incidence of sporotrichosis in this region ranged from 48 to 60 cases per 100,000 persons and was highest among children aged 7-14 years, approaching 1 case per 1000 persons. Sporotrichosis is a significant mycosis in the rural highlands of Peru, with an incidence exceeding those of other invasive mycoses in individuals without human immunodeficiency virus infection.