A three-generation family is presented in which several relatives died from or are affected by idiopathic dilated cardiomyopathy (IDC). The transmission pattern is autosomal dominant. Although familial instances of IDC have been reported the proportion of familial cases tends to be underestimated. Moreover, different transmission patterns have been associated with IDC. This report adds further evidence to the fact that an autosomal dominant form of IDC clearly exists and that early screening of asymptomatic first degree relatives is mandatory for accurate genetic counselling of patients and their family members.