Visceral leishmaniasis (VL) is a severe vector-borne parasiti disease, endemic in various tropical countries but also in the Mediterranean Basin. We report the case of a 64-year-old immunocompetent male, who presented with jaundice, fever, splenomegaly, hyperferritinaemia and cytopenias after a recent trip to Portugal. Clinical and biochemical findings were consistent with the diagnosis of an acute cholestatic hepatitis and haemophagocytic lymphohistiocytosis (HLH), secondary to visceral Leishmania infantum which was later confirmed by bone marrow examination. Despite adequate therapy with liposomal amphotericin B, the disease and HLH relapsed. Retreatment with higher dosing was eventually successful. This case highlights the risk of acquiring VL in southern Europe and its possible unusual presentations, causing diagnostic delay, as well as its complex management that requires appropriate multidisciplinary communication.