Leishmaniasis caused by Leishmania infantum is endemic in the south of France. Here, we report a case of isolated pulmonary leishmaniasis in a patient with chronic obstructive pulmonary disease. In November 2023, the patient underwent a transbronchial biopsy, which resulted in a histopathological diagnosis of leishmaniasis. This finding was confirmed by polymerase chain reaction (PCR) testing targeting Leishmania species on the biopsy tissue and positive serology, although the results of PCR testing on peripheral blood were negative. These results supported a diagnosis of isolated pulmonary leishmaniasis. Additionally, no clinical or biological indicators of visceral leishmaniasis were identified. Atypical forms of leishmaniasis are rare and often lead to delayed diagnosis. In cases with unusual clinical presentations and a history of exposure in an endemic area, evaluation for leishmaniasis should include a comprehensive assessment of all potentially affected organs, regardless of the patient's immune status.