Neoplasms of the cardiovascular system are rare pathologies associated with non-specific signs and symptoms. Cardiac neoplasms should be considered when treating cardiac arrhythmia, Hiss-Purkinje conduction system dysfunction and ischemic syndromes. Tumours of the cardiac muscle are rhabdomyosarcomas that develop frequently in the left chambers, mainly the left ventricle. Among endovascular tumours, the most common one is the myxoma of left atrium and its variant forms. Metastatic tumours of the heart, among them the malignant melanoma, affect the right ventricle more commonly (anterior wall and septum, with invasion of the left ventricle), in the nondisseminated form. Late diagnosis results in high morbidity and mortality rates whether surgical treatment is given or not. The authors describe the case of a right ventricle melanoma patient, comparing the diagnostic approach and treatment with published data. The treatment of choice of cardiac muscle malignant tumours (both primary and metastatic) is non-conclusive according to our literature review. Due to the low incidence of this pathology, the authors suggest additional meta-analytic studies should be performed to establish diagnostic and therapeutic criteria for the malignant tumours of the heart.