Leishmaniasis is a neglected tropical disease caused by protozoa of the genus Leishmania, transmitted by infected Phlebotomine sandflies. The disease is endemic in tropical and subtropical regions, with significant geographic variations in epidemiology and clinical presentation. Visceral leishmaniasis (VL) is predominant in South Asia and East Africa, while cutaneous leishmaniasis (CL) is highly prevalent in the Eastern Mediterranean and Latin America. Mucocutaneous leishmaniasis (ML), a severe and potentially disfiguring form, is mainly associated with L. braziliensis, L. panamensis, and L. guyanensis in South America. Oral involvement in ML often presents as destructive ulcerative lesions affecting the palate, tongue, and buccal mucosa, frequently mimicking malignancies and other granulomatous infections. Histopathological examination typically reveals hyperplastic or ulcerated epithelium, a dense chronic inflammatory infiltrate rich in histiocytes and a variable number of multinucleated giant cells. Immunohistochemistry and polymerase chain reaction (PCR) assays improve diagnostic accuracy in cases with scarce parasites. Treatment varies, and pentavalent antimonials remain the first-line therapy, though liposomal amphotericin B and miltefosine are preferred alternatives, particularly for ML and VL. Early diagnosis and tailored treatment strategies are critical for improving patients’ outcomes and mitigating disease burden.