Lamin A/C gene (LMNA) associated cardiomyopathy is a form of dilated cardiomyopathy with poor prognosis and high mortality, and a rapid evolution toward end-stage heart failure and malignant ventricular arrhythmias associated with increased risk of sudden cardiac death. It is transmitted in a autosomal dominant manner and is characterized by age-dependent high penetrance and variable expression. Screening of first degree relatives of proband patients by means of clinical evaluation, electrocardiogram, echocardiography and genetic analysis is useful for the early diagnosis of the disease. Drug therapy and non-pharmacological measures in the early stages of the disease seem to improve the prognosis of these patients.