Ankylosing spondylitis (AS) is a chronic inflammatory condition that most commonly affects the axial skeleton. The most common cardiac manifestation in patients with AS is the aortic root and valve disease, followed by conduction and rhythm abnormalities, decreased coronary flow reserve, myocardial infarction, and diastolic dysfunction. However, the presence of systolic dysfunction has been less described in patients with AS. Herein we present two cases of idiopathic dilated cardiomyopathy in patients with AS. These patients were noted to have an improvement of their ejection fraction following treatment of AS. Clinical and echocardiographic improvement on anti-inflammatory treatment might be a clue to the inflammatory nature of this myocardial problem, and further investigations to study the issue is required.