Cutaneous Leishmaniasis is the most prevalent type of leishmaniasis. Iran is among the first six countries in the world with the highest annual incidence of cutaneous Leishmaniasis. Non-caseating granulomatous inflammatory cutaneous reaction is the most common pattern of its skin lesions. The current article presents the case of a 36-year-old man with a history of polyarteritis nodosa vasculitis with extensive caseating granulomatous lesions who was diagnosed with cutaneous leishmaniasis. Caseating granulomatous can present in cutaneous Leishmaniasis; thus, it is recommended to consider leishmaniasis in the diagnosis of this histological pattern, especially in autoimmune patients before starting immunosuppressive treatment.