Chloroquine (Hydroxychloroquine)-induced cardiomyopathy is a rare but potentially life-threatening condition. Cessation of the culprit drug, along with aggressive afterload reduction therapy, has been associated with halting of disease progress and even improvement in patients' clinical and histologic status. Echocardiography is a fundamental tool in the identification and assessment of patients with cardiomyopathy, with particular utility in the detailed assessment of biventricular systolic and diastolic function. It also provides an objective and non-invasive means of assessing treatment response. We present a case of a 51-year-old woman with hydroxychloroquine-induced restrictive cardiomyopathy and correlate clinical, echocardiographic and anatomic pathologic findings both at initial presentation and following treatment.