Visceral leishmaniasis is a serious zoonosis which has very rarely been diagnosed in Norway. We report a case of visceral leishmaniasis in a Norwegian patient, and present an up-to-date review of the disease. We conclude that this disease should be considered even in Scandinavian patients if the appropriate symptoms and signs are present and there has been possibility of exposure. Simple blood tests and serum electrophoresis are of considerable value. If possible, the protozoa should be demonstrated by microscopy of bone marrow aspirate. We also discuss the identification of the parasites in bone marrow biopsy, which should be performed in pancytopenic patients. The diagnosis should be confirmed by culture or a serological test.