Pseudoaneurysm of the mitral–aortic intervalvular fibrosa (P-MAIVF) is an infrequent but potentially life-threatening condition. Both transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) can detect P-MAIVF with sensitivity rates of 43 and 90 %, respectively. The typical finding of echocardiography is a pulsatile echo-free sac that expands in systole and collapses in diastole. Our review comprises 166 patients with P-MAIVF, including eight cases in our hospital and 158 cases from the literature. P-MAIVF is often associated with infection or surgical trauma. While it is likely to maintain an asymptomatic course, symptoms of shortness of breath, heart failure, valvular disease, chest pain, endocarditis, and cerebrovascular events are common clinical presentations. The recommended treatment is surgery. However, conservative therapy is an alternative approach for high-risk patients or when surgical treatment is refused. With the increasing incidence of cardiac surgery and infective endocarditis, a likely increment in the new diagnosis of pseudoaneurysm is expected.