Leishmaniasis is a parasitic disease caused by an intracellular protozoan that belongs to the genus Leishmania and is transmitted by a phlebotomine sandfly. In Southwest Europe, including Portugal, cutaneous leishmaniasis is considered a rare disease of unknown or underestimated prevalence. Leishmania infantum is the only species identified as responsible for the autochthonous cases.We report the case of a 66-year-old man with an erythematous, painless plaque on the mid face region, accompanied by nasal obstruction with 9 months of evolution. The initial diagnoses were: lymphoma, subcutaneous mycosis, Wegener's granulomatosis, and lupus vulgaris. The diagnosis of leishmaniasis was based on histopathology findings and identification of L. infantum by DNA based methods. Blood cultures, abdominal ultrasound and myelogram ruled out systemic involvement. The patient was treated with intravenous meglumine antimoniate (20 mg per kg/day) for four weeks, without major side effects.We emphasize the importance of this case because human cutaneous leishmaniasis has rarely been diagnosed in Portugal and some cases are atypical, such as the situation herein described.