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Atrial myxomas are the commonest benign primary tumours of the heart. They are generally 2 to 6 cm in size. Depending on their size and site may result in mitral valve obstruction which may lead to pulmonary hypertension. Clinical symptoms may suggest the presence of a myxoma but echocardiography is the mainstay of diagnosis and confirmation is by histopathology. A well-formed, organized thrombus is a common differential. The report of an unusually large left atrial myxoma that occurred in a 54-year-old male causing mitral valve obstruction and pulmonary hypertension is presented here.