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Introduction: Fever of unknown origin (FUO) has different definitions depending on the context. The classic definition is: fever of 38.3 °C for >3 weeks that remains undiagnosed after a series of laboratory and diagnostic imaging studies. In the approach to patients with hematologic malignancies and fever of unknown origin, rare disease presentations should be considered as possible causes. Case presentation: We describe the case of a 27-year-old woman with a history of acute myeloid leukemia who presented with febrile neutropenia after a course of chemotherapy. Fever persisted for several weeks despite treatment for isolated germs and ruling out neoplastic activity. After presenting with abdominal pain and jaundice, an abdominal MRI was performed with findings suggestive of hepatosplenic candidiasis. The patient was started on treatment with fluconazole and prednisolone, which resolved her symptoms. One year later the patient still has hepatic lesions so she continues her treatment. Conclusion: Hepatosplenic candidiasis is the most common form of chronic disseminated candidiasis. The presence of fever after recovery from absolute neutropenia should always raise suspicion of hepatosplenic candidiasis. The present case clearly illustrates the recommendation to guide the workup of a patient with FUO based on the presentation of potentially diagnostic clues.

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