Chediak-Higashi Disease (CHD) is an ultra-rare genetic disease characterized by oculocutaneous albinism, bleeding diathesis, congenital immunodeficiency, hemophagocytic lymphohistiocytosis (HLH), neurodevelopmental deficits and neurodegeneration after the first decade of life.1 CHD is caused by biallelic variants in the lysosomal trafficking regulator gene, LYST. The gene discussed is LYST; the disease is coronary artery disorder.