Visceral leishmaniasis (VL) is a vector-borne parasitic disease targeting tissue macrophages. It is among the most neglected infectious diseases. Classical manifestations of VL include chronic fever, hepatosplenomegaly, and pancytopenia. Most cases can be detected through serologic and molecular testing. Although therapy has historically relied on antimonials, newer therapeutic options include conventional or liposomal amphotericin B, paromomycin and miltefosine. Coinfection with human immunodeficiency virus (HIV) is increasingly reported and comes with additional diagnostic and therapeutic challenges. This article provides an up-to-date clinical review of VL focusing on clinical presentation, diagnosis, management, and issues related to HIV coinfection.