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Background Cerebral cardiac embolism accounts for an increasing proportion of ischemic strokes and transient ischemic attacks. Calcified cerebral emboli are rare and mostly iatrogenic secondary to heart or aorta catheterization. However, spontaneous cerebral calcified embolism in the case of calcified aortic valve is very rare and there are less than 10 case reports in the literature. And a more interesting fact is that such an event, in the context of calcified mitral valve disease, has never been reported, at least to our knowledge. We are reporting a case of spontaneous calcified cerebral embolism revealing a calcified rheumatic mitral valve stenosis. Case presentation We report a case of a 59 year-old Moroccan patient, with a history of rheumatic fever at the age of 14 and no history of recent cardiac intervention or aortic/carotid manipulation, who was admitted to the emergency department after a transient ischemic attack. Physical examination at admission found normal blood pressure of 124/79 mmHg and heart rate of 90 bpm. A 12-lead electrocardiogram showed an atrial fibrillation, no other anomalies. Unenhanced cerebral computed tomography imaging was performed, revealing calcified material inside both middle cerebral arteries. Transthoracic echocardiography was performed, showing severe mitral leaflets calcification with a severe mitral stenosis, probably due to rheumatic heart disease. Cervical arteries Duplex was normal. A vitamin K antagonist (acenocoumarol) was prescribed, targeting an international normalized ratio of 2–3 and mitral valve replacement surgery was performed using mechanical prosthesis. Short- and long-term health, with a 1-year follow-up, were good and the patient did not experience any stroke. Conclusion Spontaneous calcified cerebral emboli secondary to mitral valve leaflet calcifications is an extremely rare condition. Replacement of the valve is the only option to prevent recurrent emboli and outcomes are still to be determined.