Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy characterized by deep increased trabeculation in one or more segments of the ventricle. The apical segment of the left ventricle is most commonly affected, but left ventricular basal segment, biventricular involvement or right ventricle predominance have also been described. While some neuromuscular anomalies and myopathies had been described in systemic sclerosis patients, coexistence of chronic inflammatory disorders and NCM is unclear. This paper presents a case of biventricular NCM with severe systolic and diastolic dysfunction in a 40-year-old female diffuse cutaneous systemic sclerosis patient.