Hemophagocytic lymphohistiocytosis (HLH) is a systemic inflammatory syndrome characterized by heightened activation and proliferation of nonmalignant macrophages and excessive cytokine release. Whereas acute kidney injury is common in this syndrome, direct glomerular involvement by activated histiocytes is very rare. We present the case of a man in his 20s who presented with fevers, malaise, flank pain, anemia, thrombocytopenia, severe acute kidney injury, and proteinuria. A kidney biopsy revealed histiocytic glomerulopathy and subacute thrombotic microangiopathy, and he was diagnosed with HLH. Recovery of kidney function occurred following steroid therapy. A review of kidney involvement by HLH is provided.