In most patients with basal ganglia calcification no disturbance of calcium metabolism is present,We present four patients with basal ganglia calcification. Two suffered from a secondary hypoparathyroidism following a complicated strumectomy years ago, one had an Alport-Syndrome and hypoparathyroidism. Her mother showed basal ganglia calcification and an abortive Alport-Syndrome as well, but no hypoparathyroidism.