Churg-Strauss syndrome (CSS) is a rare small- or intermediate-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, necrotizing granulomas, and hypereosinophilia. In this report, we describe the case of a 35-year-old woman who, during her third trimester of pregnancy, developed dyspnea and, after delivery, severe cardiac failure which required heart transplantation. Diagnosis of CSS was made after performing a myocardial biopsy. We have also undertaken a review of the English-language literature regarding previously reported cases of pregnancies in women suffering from Churg-Strauss syndrome with particular attention to those patients with cardiovascular involvement.