<h4>Purpose</h4>Familial retinal arterial macroaneurysm (FRAM) is a rare genetic disorder caused by a gene mutation in the insulin-like growth factor binding protein 7 (IGFBP7).<h4>Observations</h4>We report a 30-year-old male with FRAM and IGFBP7 gene mutation who presented with an acute coronary syndrome (ACS). This evidence concerns the gene IGFBP7 and acute coronary syndrome.