PURPOSE: Primary HIV-1 infection (PHI) can present with protean clinical manifestations. We report a rare presentation of PHI that underscores that a high index of suspicion is required for diagnosis of PHI. METHODS: We report on a 54-yearold previously healthy woman of African descent who presented with sudden-onset nephrotic-range proteinuria and acute kidney injury (AKI) requiring hemodialysis in the setting of febrile multiple organ dysfunction syndrome. Both the epidemiological and clinical features initially pointed to imported Lassa fever, but this was ruled out. She was eventually diagnosed with PHI. We reviewed the literature for other patients who presented with PHI and AKI requiring hemodialysis. RESULTS: Kidney biopsy evaluation, including conventional and electron microscopy, revealed minimal change disease (MCD) and diffuse tubular damage leading to AKI. To date, MCD has not been reported to be associated with PHI and severe AKI. A literature search revealed six additional cases of severe PHI-associated AKI requiring hemodialysis. In four cases, severe rhabdomyolysis with tubulotoxic myoglobinuria played the primary causative role, while in one case each AKI was associated with HIV-associated nephropathy (HIVAN) and hemolytic uremic syndrome, respectively. CONCLUSIONS: Severe AKI requiring hemodialysis is a rare manifestation of PHI and may be associated with several conditions, most commonly PHI-associated rhabdomyolysis with tubulotoxic myoglobinuria. Severe AKI in PHI may also occur as a complication of MCD manifesting with nephrotic-range proteinuria. PHI should be considered in the differential diagnosis in patients presenting with severe proteinuria and AKI in the setting of febrile multiple organ dysfunction syndromes, including hemorrhagic fever diseases.