In acromegaly, dilated cardiomyopathy is rare. Indeed, arterial hypertension, coronary artery disease and hypertrophic cardiomyopathy are more common. The authors report a case of a 42-years-old man with dyspnea (class III NYHA) and palpitations. The cardiac echography showed a dilated cardiomyopathy. The investigations revealed an acromegaly as an etiology. Diagnosis was based on patient morphotype, a high level of growth hormone (GH) and a pituitary adenoma detected by magnetic resonance imagery. This case is characterized by the absence of arterial hypertension and hypertrophic cardiomyopathy. The coronary angiography was normal. Consequently, in this particular case, dilated cardiomyopathy seems specific to acromegaly.