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Cardiac myxomas (CM) are the most common type of primary cardiac tumours in adults, which have an approximate incidence of up to 0.2% in some autopsy series. The purpose of this review is to summarise the literature on CM, including clinical presentation, differential diagnosis, work-up including imaging modalities and histopathology, management, and prognosis. CM are benign neoplasms developed from multipotent mesenchyme and usually present as an undifferentiated atrial mass. They are typically pedunculated and attached at the fossa ovalis, on the left side of the atrial septum. Potentially life-threatening, the presence of CM calls for prompt diagnosis and surgical resection.