Visceral leishmaniasis (VL) is a well recognized opportunistic infection in patients with HIV-1 infection, which may occasionally present with atypical features. We describe two patients with advanced HIV-1 infection (CD4<100/ mm3) in whom visceral leishmaniasis presented with atypical features, and their response to therapy. Atypical features of visceral leishmaniasis in the two infected patients include absence of fever, dissemination to the duodenal mucosa and to the skin as xanthoma-like lesions. Therapy and secondary prophylaxis remain unsatisfactory, and studies to evaluate combinations of amphotericin B and immunotherapy are needed.