A young patient underwent a screening electrocardiogram (EKG) that suggested apical hypertrophic cardiomyopathy. Serial investigation with echocardiography showed a well-defined hyperechogenic mass involving the interventricular septal. To better define the lesion extension three-dimensional (3D) echocardiography was done and it demonstrated a mass invading the septal myocardium, involving the major part of the muscular portion. The findings were highly suggestive of a cardiac fibroma. A cardiac magetic resonance image (MRI) was also compatible with this diagnosis. In our case, 3D echo showed a high accuracy, proving to be a useful tool to determine the anatomy of the lesion, complementary to MRI, guiding best management strategy.