The incidence of infantile visceral leishmaniasis is currently increasing, at least in the Mediterranean region. Most cases seen in France occur on the Mediterranean coast or are imported from Africa. However, contamination in other regions of France is not an exceptional occurrence and may raise diagnostic problems. The parasite reservoir is the dog population in which the prevalence of Leishmania infection is particularly high in the Provence and Cévennes regions. Both teenagers and young children may be affected by this disease, whose clinical manifestations may be misleading. The typical symptomatic triad, i.e., anemia-fever-enlarged spleen, may be incomplete, especially as a result of the intermittent character of the fever. Patients may remain afebrile for long periods. Diagnosis rests on demonstration of the parasite in bone marrow specimens (several biopsies are often required) or in the spleen. Bacteriologic studies using a special medium are helpful. Serologic tests are sensitive and specific but often become positive only late in the disease. Management still rests on pentavalent antimonial compounds. Advances have been made in the understanding of the toxic effects and rules for optimal use of these drugs. Improved insight into the parasite's biology may result in use of new forms of treatment; allopurinol is at present the only recent addition to the armamentarium which has been proved effective in humans when given in combination with an antimonial compound.