Cardiovascular disease in pregnancy contributes to a significant proportion of death worldwide. Though pregnancy-associated myocardial infarction and aortic dissection are the common causes of adverse cardiac events in developed countries, rheumatic heart diseases continue to be the important reason for cardiovascular morbidity and mortality in developing countries. The risk of adverse cardiac outcome is dependent on the type and severity of valvular abnormality, functional status, left ventricular function, and pulmonary arterial pressure. Managing a pregnant woman with a mechanical heart valve prosthesis is challenging because of the difficulty in achieving optimal anticoagulation in the presence of hypercoagulability. Mitral valve thrombus is a life-threatening event and women can present with acute heart failure or thromboembolic events. We report successful management of a 26-year-old primigravida with rheumatic heart disease diagnosed to have huge thrombus on mechanical prosthetic mitral valve presented with acute heart failure at 36 weeks. She received multidisciplinary care and underwent concurrent cesarean section followed by thrombectomy under cardiopulmonary bypass. She had a good recovery following surgery and the complexity surrounds the management merit the presentation with a review of management strategies for a women with mechanical prosthetic heart valve in pregnancy.