According to current definition, peripartum cardiomyopathy (PPCM) is a rare disorder in which left ventricular dysfunction and symptoms of heart failure occur in the last month of pregnancy. It has been reported that the incidence of PPCM is 1 in 3,000–4,000 live births. The pathogenesis is poorly understood, however, infectious, immunologic, and nutritional causes have been hypothesized. Clinical presentation includes usual signs and symptoms of heart failure, and unusual presentations such as thromboembolism. Diagnosis is based upon the clinical presentation of congestive heart failure and the objective evidence of left ventricular systolic dysfunction. Early diagnosis and initiation of treatment are essential to optimize pregnancy outcome. Patients with systolic dysfunction during pregnancy are treated similar to patients who are not pregnant. The mainstays of medical therapy are digoxin, loop diuretics, sodium restriction and afterload reducing agents (hydralazine and nitrates). Due to a high risk for venous and arterial thrombosis, anticoagulation with subcutaneous heparin should be instituted. Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers should be avoided during pregnancy because of severe adverse neonatal effects. Effective treatment reduces mortality rates and increases the number of women who fully recover left ventricular systolic function. The prognosis is poor in patients with persistent cardiomyopathy. Subsequent pregnancies are often associated with recurrence of left ventricular systolic dysfunction.