Aims Primary cardiac tumours are rare; most are benign, and of these, around half are myxomas, often located in the left atrium. Clinical presentation is variable. Ischaemic stroke is a rare, although real and potentially fatal, complications of cardiac myxomas. Methods and results We present a case of a 51-year-old man, ex- smoker, obese, with a history of hypertension and COPD, presented to our emergency department with right-sided hemiplegia and aphasia caused by ischaemic stroke. Brain computed tomography revealed cerebral perfusion deficit. The patient was underwent intravenous thrombolytic strategy and intubated transferred in Reanimation. TTE showed a large left atrial mass attached to the interatrial septum, with a friable appearance, suggestive of myxoma. The 2D and 3D TEE detected a giant space-occupying mass (60 × 20 mm diameter) in the left atrium, coral-like, and with a friable appearance, the most apical portion prolapsed into the left ventricle during diastole, causing fixed obstruction to flow in the left ventricular inflow tract. Computed tomography angiography (CTA) confirmed the giant mass in the left atrium. In view of the risk of imminent embolization, the emergent surgical excision of the tumour was performed. The tumour and its neck, including part of the atrial septum, were fully resected and the atrial septum was directly closed. Subsequent histopathological findings confirmed the diagnosis of atrial myxoma. Post-operative echocardiography showed no residual tumour. Symptomatic cardiac myxoma may present with one of the three classic clinical presentations of the Goodwin’s triad, which include intracardiac obstruction, constitutional symptoms, and embolism. The obstructive pattern mimics mitral or tricuspid valve disease and results from atrioventricular valve obstruction. Constitutional or systemic manifestations include fatigue, fever, weight loss, arthralgia, myalgia, erythematous rash, and laboratory findings such as anaemia and elevated ESR, CRP, and globulins. Systemic embolization from myxoma occurs in around a third of cases. Of these, the most serious is cerebrovascular embolism, which may result in cerebral stroke. In addition, embolization of coronary arteries, kidneys, intestines, and extremities can also occur. The tumour size, location, and macroscopic appearance, along with mean platelet volume and platelet count, are closely associated with embolic events. Echocardiography remains the method of choice for diagnosis and morphological characterization of myxoma. Two patterns have been established by echocardiography: round, with a solid appearance and a firm surface, and polypoid, with an irregular outline and a friable surface. The incidence of systemic embolization is higher in those with an irregular and friable surface, as well as in polypoid tumours and those that prolapse into the ventricle. Once a diagnosis of myxoma is established, surgical resection is the only effective treatment and should be performed immediately, in view of the risk of embolic complications. Conclusions With this case report, we stress the fundamental importance of echocardiography in new onset of neurological deficit to prevent potentially fatal outcomes.