Visceral leishmaniasis (VL) is endemic in areas bordering the Mediterranean Sea (Spain, Italy, France, Greece, Morocco, Tunisia) where it is caused by Leishmania infantum and it is transmitted by the bite of hematophagous sandfly belonging to Phlebotomus spp.; dog constitutes the main reservoir of the infection. In comparison with the past, when VL was typically observed more frequently in children, the current ratio of childhood to adult cases is approximately 1:1. The onset of the disease is characterized by a non-specific initial symptomatology; fever, pallor and splenomegaly are always present. Pancytopenia is present very often; the laboratory diagnosis is established by serological tests (indirect fluorescent-antibody assay, immunoassay test, indirect hemagglutination assay) and by demonstration of Leishmania parasites by microscopy, culture or polymerase chain reaction (PCR) in the bone marrow aspirates. The use of PCR performed on peripheral blood has been reported to be highly sensitive for the diagnosis and the follow up of children with VL. Pentavalent antimonial drugs have been used for many decades as standard treatment for VL; in Italy liposomal amphotericin B (AmBisome) is nowadays considered the first-line treatment for VL.