The clinical presentation and imaging of venous congestive encephalopathy (VCE) can mimic several neurological conditions, making the diagnosis challenging. We report a patient with end stage renal disease on dialysis who presented with a right occipital infarction. The patient developed progressive encephalopathy and increased intracranial pressure. Extensive imaging, electroencephalography and serum analysis did not explain the cause of his infarction and progressive neurological deterioration. Finally, cerebral angiography and venography demonstrated severe generalized VCE due to arterial shunting from a right upper extremity arteriovenous graft (AVG) and an occluded right innominate venous trunk. The right arm shunt resulted in severe cerebral venous hypertension due to ipsilateral occlusion of the innominate venous trunk. After the AVG was repaired, the cerebral venous hypertension resolved and the patient returned to baseline.