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Mitral annular disjunction (MAD) is the atrial displacement of the mitral valve (MV) hinge point, especially along the posterior mitral leaflet, which leads to inhomogeneous blood flow into the left ventricle, causing chronic fibrotic changes, malignant arrhythmias, and even sudden cardiac arrest. Some studies suggest that MAD is a part of normal heart morphology; however, the origin is still controversial. MAD commonly occurs with MV prolapse and myxomatous degenerative MV disease. In almost 20% of cases, MAD can occur independently as well. The prevalence of MAD in normal hearts varies from 8.6% to 96%, depending on the imaging modality and the cutoff used to define MAD. Transthoracic echocardiography is often the initial screening test, but the low sensitivity of transthoracic echocardiography to identify MAD makes it easy to miss the diagnosis altogether. More advanced imaging, especially cardiac MRI, is the gold standard for diagnosing MAD and risk stratification. MAD is an independent predictor of malignant arrhythmia. Among patients with MAD, risk stratification is based on the age at diagnosis, previous syncopal attacks, premature ventricular contractions, papillary muscle fibrosis, and longitudinal disjunction distance. Most asymptomatic patients are managed conservatively; however, radiofrequency ablation should be considered in patients with high-risk or symptomatic MAD due to the risk of ventricular arrhythmias and sudden cardiac death.