Hemophagocytic syndrome (HPS), a rare and life-threatening disease, is characterized by hyperactivation of the immune system that causes hypercytokinemia and potential multiorgan failure. Acute kidney injury is the most common kidney manifestation of HPS and is generally considered a poor prognostic factor. Glomerular involvement is uncommon and usually manifests as either podocytopathy with collapsing glomerulopathy or thrombotic microangiopathy. We report a rare case of severe histiocytic glomerulopathy in a patient with HPS who presented with acute kidney injury and proteinuria.