Myxoma, the most common cardiac tumor is known to cause mitral valve obstruction and right heart failure with pulmonary hypertension. Patients with this condition often present with dyspnea, peripheral edema, and other signs of right heart failure. Pulmonary hypertension secondary to mitral valve obstruction presents unique difficulties in diagnosis as opposed other secondary causes such as thromboembolic pulmonary hypertension. Multimodality imaging with MRI, CT, and echocardiography can help resolve this dilemma. This case report serves to elucidate the important role of multimodality imaging in the management of patients with such a presentation. We also demonstrate in our case report how surgical removal can be curative by relieving mitral valve obstruction and thereby reversing pulmonary hypertension.