Background: Visceral leishmaniasis (VL) is endemic in large areas of the tropics, the subtropics, and the Mediterranean basin. Besides classical VL presentation, exceptional cases of a limited form of VL have been reported. Here we describe the challenges of diagnosis and management of this intriguing entity. Case summary: A 25-year-old French Caucasian man presented with marked asthenia that had lasted 6 months and was strictly isolated except for a 2-cm left cervical lymphadenopathy. The rest of the clinical examination and extensive biological exploration were unremarkable.Histological examination of the cervical lymphadenopathy showed a reactive lymphoid hyperplasia with granulomatous organization associated with small particles in the cytoplasm of epithelioid histiocytes and giant cells evocative of Leishman-Donovan bodies. Polymerase chain reaction (PCR) performed on the tissue confirmed the presence of Leishmania donovani/infantum DNA. Direct examination of a bone marrow aspiration, together with blood and bone marrow PCR, did not find other evidence for VL. Serology for leishmaniasis was unreactive. Extensive work-up for other causes of granulomatous lymphadenitis was negative. A diagnosis of localized leishmanial lymphadenopathy was made. Intravenous liposomal amphotericin B (20 mg/kg in five infusions) was initiated and well tolerated. Asthenia disappeared promptly and the patient fully recovered. Conclusion: Localized lymph node enlargement because of leishmanial infection should be included in the differential diagnosis of lymphadenopathy of unknown origin in patients who stayed or visited, even a long time ago and for a short period, endemic areas for leishmaniasis such as the Mediterranean basin. Fine-needle aspiration cytology and/or PCR for Leishmania sp of the lymphadenopathy might contribute to the diagnosis. A low-dose liposomal amphotericin B treatment might be effective, and deserves further study.