Lamin A/C is a major constituent of the nuclear lamina, the proteinaceous meshwork underlying the inner nuclear membrane. Laminopathies are a group of diseases with heterogeneous clinical presentation. Lamin A/C mutations are a well-established cause of dilated cardiomyopathy. In our case, a novel mutation of lamin A/C presented in the typical form of cardiolaminopathy with ventricular tachycardia and mild myocardial dysfunction in an apparently healthy, middle-aged individual.