IntroductionHemophagocytic lymphohistiocytosis (HLH) is a potentially lethal immune system dysregulation in children and adults. Failure to properly inhibit the immune response leads to constant and excessive activity of the cytotoxic T-cells, natural killer (NK) cells, and macrophages. Inflammatory reactions caused by the intense activity of the cellular immune system and cytokine storm in tissues lead to multiple organ failure in this disease. HLH is categorized as primary, which defines as the presence of a predisposing genetic mutation in the immune system, and reactive to an infectious, inflammatory, or malignant trigger. The diagnosis is challenging as it has no clinical or laboratory pathognomonic features. Fever, organomegaly, liver dysfunction, cytopenias, coagulopathy, hemophagocytosis, and neurologic dysfunction are common manifestations of HLH.Ocular involvement is relatively rare in HLH. Unilateral panuveitis, Purtscher retinopathy, trabecular meshwork involvement, and choroidal infiltration with secondary extension to the retina and optic nerve head (ONH) have been reported previously.This report aims to introduce a child with HLH and bilateral ONH infiltration.