Purpose: To report clinical and molecular findings in a family with a novel laminA/C (LMNA) mutation.Methods: A 41 year old man survived a heart arrest due to monomorphic ventricular tachycardia-VT (with right bundle branch block morphology), his magnetic resonance imaging (MRI) showed mildly depressed biventricular systolic function and a defibrillator (ICD) was implanted. His four living first degree relatives underwent familial screening including physical examination, blood analyses, ECG, echocardiography; MRI, Holter ECG and exercise testing were also performed in affected individuals. Based upon clinical findings LMNA gene was sequenced in the proband followed by family cascade screening.Results: The proband exhibited a motor sensory neuropathy and control echocardiography showed normal biventricular function. One of his sisterʼs died because of severe cardiac failure with nearly normal biventricular function. The probandʼs father and 4 of his siblings died suddenly at the age of 50-65 with previous mild heart failure symptoms, and another one had a pacemaker implanted before dying. Clinical evaluation was normal in the probandʼs mother and one sister. The brother and the other sister showed normal ventricular dimensions, preserved systolic function, atrial tachyarrhythmias, significant sinus pauses and scarce runs of non-sustained VT during exercise testing. CK was not elevated in any of the evaluated individuals. LMNA sequencing showed a novel missense mutation F237S in heterozygosis in the proband. Cascade screening confirmed its presence also in the two siblings with atrial arrhythmias. An ICD was recommended in the two affected probandʼs siblings.Conclusions: Herein we present a family with a novel missense mutation causing a high rate of sudden death, cardiac failure, preserved ventricular function, atrial and ventricular arrhythmias.