Beta-thalassemia major is a complex medical problem found worldwide. Endocrinopathies are some of the most frequent dysfunctions found in these patients. Iron overload in different organs is responsible for multiple endocrine complications particularly in the absence of adequate chelation therapy. One of the most prevalent endocrine complications of thalassemia major is hypoparathyroidism. It can cause cerebral calcifications in the basal ganglia but seldom outside of the extrapyramidal system. There are few studies about intracerebral calcification due to hypoparathyroidism in patients with thalassemia major. We report the case of a 14 year-old girl who came to our Center with the chief complaint of a generalized tonic-clonic seizure. The patient was known to have beta-thalassemia major since she was 9 months old. Computerized tomographic scan of the brain was done which showed diffuse intracranial calcifications in deep white matter, posterior fossa, basal ganglia and both thalami. Laboratory and neuroimaging assessments revealed the diagnosis of hypoparathyroidism. We strongly recommend periodic assessment and tight control of serum calcium level in all patients with betathalassemia major. Prompt treatment with oral calcium supplements and an active form of vitamin D can prevent hypoparathyroidism and its neurologic complications. Comprehensive evaluation and treatment of other endocrinopathies in accordance with hypoparathyroidism is suggested.