We describe the unusual case of a 63-year-old woman with a history of arterial hypertension who presented a sudden weakness of the lower limbs followed by mutism, akinesia and dyspraxia. Magnetic resonance images showed a bilateral medial frontal infarction. Digital subtraction angiography documented a right azygous anterior cerebral artery with severe stenosis in its sub-callosal tract; the left anterior cerebral artery showed mild hypoplasia with only sub-frontal and fronto-polar branches. No embolic source was documented. Afterwards the patient presented a gradual and partial recovery of both motor and cognitive functions.