Lutembacher syndrome (LS) is a rare cardiac abnormality characterized by any combination of a congenital or iatrogenic atrial septal defect (ASD) and a congenital or acquired mitral stenosis (MS). Clinical features and hemodynamic effects of LS depend on the balance of effects of the MS and the ASD. Prognosis is influenced by several factors [pulmonary vascular resistance, right ventricle (RV) compliance, size of ASD and MS severity] but the occurrence of secondary pulmonary hypertension and congestive heart failure is commonly associated with poor outcome. Echocardiography remains the gold standard for diagnosis and evaluation of LS. Timely diagnosis is critical for modifying the natural course, by allowing patients to benefit from currently available percutaneous trans-catheter therapies with favorable effects on the outcomes. This article is a review of published literature on the current diagnostic and therapeutic modalities for LS, focusing on the pivotal role of echocardiography as the key diagnostic tool. Clinical suspicion of LS should prompt extensive investigation with non-invasive and where possible, invasive technics. Multicenter registers have a potential to assist the evaluation of long term outcomes of percutaneous trans-catheter therapies in patients with LS.