The article presents a clinical case of a rather rare course of hypertrophic cardiomyopathy with the transformation of a hypertrophic phenotype into a dilated phenotype against the background of the “burned-out phase” phenomenon, ventricular and supraventricular rhythm disturbances, and multiple genetic mutations. Timely started disease-modifying therapy (quadruple therapy) for chronic heart failure led to reverse positive remodeling of the left chambers of the heart.