BACKGROUND: Leishmania infantum is the only prevalent Leishmania species in Europe and manifesting predominantly as cutaneous or visceral leishmaniasis, whereas new world species like Leishmania (L.) braziliensis are well known pathogens in mucocutaneous leishmaniasis. Mucosal leishmaniasis caused by L. infantum is a rare clinical condition with only few cases described in literature. In contrast to our case, mostly immunocompromised patients with no history of leishmaniasis are affected. CASE PRESENTATION: We describe the case of a 77-year-old German male who developed an ulcerous lesion of the tongue. As oral cancer was suspected, the patient underwent surgery. After suspected diagnosis of Leishmania spp. in histopathology, the patient was referred to our department for further diagnostics and treatment. Relapse from a cutaneous leishmaniasis acquired in Spain is likely, as L. infantum could be identified as the causative agent. The patient recovered after treatment. CONCLUSIONS: Mucosal leishmaniasis caused by L. infantum is rare and usually mistaken for malignancy. As demonstrated, it can be preceded by cutaneous leishmaniasis of the immunocompetent. Due to possible dissemination systemic treatment should be applied.