To report a case of 46-year-old male patient with structural focal epilepsy and mild cognitive impairment who presented to the emergency department with 15 days history of neurological deterioration. Simple brain CT revealed multiple brain calcifications in basal ganglia, white matter and cerebellum, along with hypocalcemia, hyperphosphatemia, hypomagnesemia and normal PTH. A diagnosis of Fahr syndrome associated with pseudohypoparathyroidism was considered. Cerebral calcinosis syndrome is defined as the accumulation of calcium in the brain parenchyma, frequently involving the basal ganglia and it's most frequent cause is pseudohypoparathyroidism.