We present a woman with a history of Gaucher disease and secondary portal hypertension. She went to hospital for treatment of resistant fever without apparent cause. A conventional study of fever of unknown origin did not show any pathology. For this reason, she was referred to our department for a PET/CT, which did not demonstrate a cause for the fever. Two months after, she was admitted again in hospital for fever recurrence. A new PET/CT showed FDG avid hypodense splenic lesions, suspicious for infection. Splenectomy was performed and histopathologic analysis demonstrated intracellular organisms compatible with Leishmania.