Kaposi sarcoma is a malignant disease that originates fromthe lymphatic system. Different epidemiological, clinical andhistopathological variants of this neoplasm have been identified.Classic Kaposi sarcoma is one of the four main clinico-epidemiologicvariants. Cutaneous lesions vary from pink patches to darkviolet plaques, nodules or polyps, depending on clinical variantand stage. Kaposi sarcoma with elephantiasis is reported in thecontext of AIDS. An 82-year-old male presented with a 2-yearhistory of progressive verrucous skin changes and non-pittingedema consistent with elephantiasis nostras verrucosa (ENV),secondary to Kaposi’s sarcoma. Past medical history, physicalexamination, lab tests and imaging ruled out common causes ofENV and anti-HIV antibody test was negative. Classic Kaposisarcoma was confirmed on biopsy. To the best of our knowledge,this study reports the first case of elephantiasis nostras verrucosain an HIV-negative patient with classic Kaposi sarcoma.