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© 2018 Indian Journal of Medical and Paediatric Oncology | Published by Wolters Kluwer Medknow Sir, Herein, we report a case of Chediak–Higashi syndrome (CHS) presented with hemophagocytic lymphohistiocytosis (HLH). A 3‐year‐old girl presented with recurrent infections, fever, and icterus. Physical examination revealed hepatosplenomegaly, bilateral cervical and inguinal lymphadenopathy, silvery gray hair, strabismus, and bilateral nystagmus. Giant granules in lymphocytes and monocytes were seen on blood smear. Bone marrow aspirate exhibited erythrophagocytosis and numerous giant granules of predominantly myeloid lineage [Figure 1a]. Examination of the hair showed an irregular distribution of large and small pigment clumps [Figure 1b]. Magnetic resonance imaging of the brain showed diffuse cerebral atrophic [Figure 1c]. The girl’s parents were consanguineous. On laboratory examination, urinary analysis was unremarkable. Hemoglobin, white blood cell count, and thrombocyte count were 7, 6 g/dL, 2300/mm3, and 76.000/mm3, respectively. Sedimentation rate was 2 mm/h (N: 0–20 mm/h), and serum triglyceride 390 mg/dL (N: 32–99 mg/dL), fibrinogen 67 ng/dL (N: 200–400 ng/dL), and ferritin was 129.000 ng/mL (N: 7–140 ng/mL) [Table 1]. Prothrombin time was 21 s (N: 11–15 s), and activated partial thromboplastin time was 62 s (N: 25–35 s).