The patient is a 61-year-old man with the complaint of progressive severe dysphagia and alalia. Patient’s symptoms started 4 years ago and were getting worse. He denied any headache, seizures, and psychiatric symptoms. Patient has hypertension for more than 10 years. Patient’s parents passed away in their sixties with unknown reason. He had an old brother and three old sisters, three of them died of unknown reason. The surviving sister is clinically asymptomatic but has calcifications on computed tomography (CT) scan, and total calcification[] score was 10. His three daughters were also asymptomatic and with normal CT scan. The pedigree chart is shown in Figure. The neurological examination was normal. Laboratory tests, including serum calcium, phosphorus, and parathyroid hormone levels, were within normal limits (calcium 2.22 mmol/L, phosphorus 0.84 mmol/L, parathyroid hormone 37.5 ng/L). Patient’s CT scan revealed multiple symmetric calcifications of bilateral basal ganglia, cerebellum, thalamus, and periventricular area. Total calcification score was 54. Genetic tests were done for the patient and his family. Excluding the dead and unreachable individuals, 7 people over two generations of the patient’s family were investigated. The patient’s genetic test showed c.1438T >G mutation and c.1271_1272 TGGTGCGC insertion mutation in exon 2 of MYORG gene. Besides the patient, his sister and two of his daughter have MYORG c.1438T>G mutation, while one daughter has MYORG c.1271_1272 TGGTGCGC insertion mutation.