Abstract Chilaiditi’s syndrome is the interposition of the small or large bowel between the inferior face of the diaphragm and the liver. This condition was identified for the first time by Demetrio Chilaiditi in 1910 and reported to have an incidence ranging between 0.025% and 0.28% in the general population. Although the syndrome is usually asymptomatic, it can cause serious complications and be a potential source of misdiagnosis for a variety of intrathoracic and intra-abdominal disorders. Here, we present a case of Chilaiditi’s syndrome with respiratory distress and acute coronary syndrome. To our knowledge, this case is the first who died due to acute myocardial infarction when hospitalized for dyspnea related to Chilaiditi’s syndrome.