Cardiac tumours are a very rare entity of tumours which comprises of both malignant and benign group. The atrial myxomas are benign cardiac tumour which occurs most commonly. This case report illustrates a diagnosis of rare atrial myxoma through its characteristic signs, symptoms, most common site of occurrence and histopathological examination assisted by radiological examination.The diagnosis is made by classical clinical presentation, histopathological examination along with 2D echocardiography to rule out the malignant activity. The clinical signs and symptoms vary with the location and size of tumour, which usually present as pulpy non-specific mass.The histopathological study corresponded with classical pattern. Since the tumour may present as an emergency in the form of symptoms mimicking sudden cardiac death and other complications like embolization, prompt resection of tumour is the mainstay treatment associated with low mortality. However, the most common complication is recurrences hence the patient should be kept in regular follow up.