The case of a 72-year-old patient affected by myasthenia gravis under pyridostigmine therapy, admitted to the hospital for the onset of oppressive thoracic pain associated with mild enzyme increase, electrocardiographic and echocardiographic disorders, is discussed. Dypiridamole/gated SPECT perfusion scintigraphy at rest evidenced reduced coronary reserve at the level of the lateral wall with apical akinesia and normokinesia of mediobasal left ventricle. On coronarography, coronary arteries were free of significant stenoses and left ventricular apical akinesia. The diagnostic approach to ischemic cardiomyopathy with the correct sequence of performed diagnostic imaging led to the presumptive identification of a rare syndrome.