In French Guiana cutaneous leishmaniasis occurs mainly in the localized form with L. guyanensis accounting for more than 90% of cases. Mucocutaneous leishmaniasis is uncommon (less than 2% of cases) with L. braziliensis accounting for all previously reported cases. The purpose of this report is to describe a case of mucocutaneous leishmaniasis due to L. guyanensis that led to diagnosis of HIV infection in a patient living in French Guiana.