Leishmaniasis caused by Leishmania infantum is endemic in the Mediterranean region. Its visceral form can present a diagnostic challenge owing to the disease's wide spectrum of clinical presentations. We describe the very atypical case of a 66-year-old male Caucasian patient with hepatopulmonary syndrome and an exceptionally rare expression of visceral leishmaniasis in a disseminated form with mucocutaneous involvement presenting as an autoimmune systemic disease.