Systemic sclerosis (SSc) is a chronic autoimmune disease that beside the skin involvement it may affect the peripheral vessels and several organs, such as the lungs, the kidneys or the heart. Cardiac impairment usually becomes symptomatic in the late stages of the disease and is associated with poor prognosis. We report the case of a 80-year-old woman patient presenting symptoms of heart failure, subsequently diagnosed with limited SSc. The cardiac function was evaluated using a combined approach based on echocardiography, cardiac magnetic resonance imaging with late gadolinium enhancement. This case shows the fatal consequences of the late diagnosis of SSc.