Acromegaly is a rare disease, mainly caused by a pituitary tumor secreting growth hormone. It is characterized by slow progression and is associated with a multisystemic involvement, being the cardiovascular system, one of the most involved, even reaching, more than 10 years ago, to represent the main cause of death. Cardiovascular complications develop as a result of elevated blood concentrations of growth hormone and insulin-like growth factor 1 (IGF-1), which exert direct and indirect effects on the endothelium, large vessels, kidney, and cardiomyocytes; causing arterial hypertension, valve disease, cardiac arrhythmia, and a specific heart disease called acromegalic cardiomyopathy. After the literature overview related to the pathophysiology, clinical manifestations, diagnosis and treatment of cardiovascular involvement, we found that from a cardiovascular point of view, patients with acromegaly can range from an asymptomatic state to severe cardiac dysfunction, being the biochemical markers and imaging studies diagnostic tools that allow assessment the degree of cardiovascular disease to provide individualized treatment. The normalization of growth hormone and IGF-1 levels improves cardiovascular parameters, and therefore its prognosis.