The diagnosis of visceral leishmaniasis (VL) is complicated and often unsuspected. Little is known of the usefulness of nuclear imaging in VL. Our objective was to describe findings seen in fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in cases of VL. We retrospectively reviewed VL cases diagnosed at Vall d’Hebron University Hospital from May 2012 to May 2018 and selected those that had an FDG-PET/CT performed. Information on procedures and details of the FDG-PET/CT features and follow-up were collected. We then systematically reviewed the literature on VL and FDG-PET/CT. Four of 43 patients diagnosed with VL had an FDG-PET/CT performed. All four patients presented diffuse splenic uptake of FDG-PET/CT. Adenopathy was not always present, and bone marrow uptake was found in two patients. A posttreatment FDG-PET/CT in one patient revealed normalization of initial findings. In the literature review, 43 of 50 cases presented similar splenic uptake in the PET/CT, being described as different patterns: “increased metabolism,” “homogeneous,” “diffuse,” “diffuse and multifocal,” “nodular,” “patchy and granular,” “subcortical,” and “compatible with lymphoma.” Other frequent findings were bone marrow uptake and adenopathies. We, therefore, conclude that FDG-PET/CT could become a useful tool for the diagnosis and follow-up of VL and that VL should be taken into account in patients with fever of unknown origin with enhanced splenic uptake in FDG-PET/CT. Differential diagnosis in these cases should be made with splenic primary lymphoma, virus infections, chemotherapy, and colony-stimulating factor therapy. Further structured studies with more cases are needed to define its diagnostic and prognostic value.