Scleroderma pericardial disease is usually silent and benign. The incidence of pericardial involvement in scleroderma is about 50% according to autopsy results, but symptomatic pericarditis manifests in about 16% of patients with diffuse scleroderma and in about 30% of patients with limited scleroderma. The clinically evident pericardial effusion is rare in scleroderma, although it can be detected in about 41% of patients with echocardiography. In majority of the patients, the pericardial effusion is small and not associated with symptoms. The pericardial effusion manifests usually after the manifestation of the other clinical and serologic features of scleroderma. A case of scleroderma is reported that presented with a large pericardial effusion, which antedated the other clinical and serologic features of scleroderma. The pericardial involvement in scleroderma is reviewed.