A 13 years old girl was admitted to the pediatric ward of hospital No 1 of Kerman university of medical sciences because of crampy pains, pins and needles sensation of the extremities and history of an episode of seizure. Her problem started one week prior to admission with pain in the leg muscles, flexion of the fingers followed by seizure. On physical examination, the prominent finding included milk hepatomegaly. Exaggeration of deep tendon reflexes and positive chvosteck & Traussau signs. Laboratory test values on admission were as follows; phosphorus 8 mg/DL, ionized calcium 2 mg/ DL and undetectable serum PTH levels. Urinalysis indicated glycosuria (++) and a mild proteinuria . Radiologic studies revealed only mild generalized osteoporosis. The patient was treated with calcitriol and calcium with the impression of hypoparathyroidism. One week later serum calcium and phosphorus were normal and the patient was discharged in good condition. One and a half months later the patient experienced ecchymotic lesions on his thigh and calf. Prothrombine time (PT) and partial thromboplastin time (PTT) were prolonged and therapy with supplementary vitamin K was started. In regard to hypoparathyroidism, hepatomegaly and coagulopathy, Wilson,s disease was considered as the etiology ,this was confirmed with low serum ceruloplasmin level, 24h high urinary excretiin of corneal Kayser- Fleischer ring. It was concluded that parathyroid insufficiency was due to Wilson,s disease,secondary to copper deposition in parathyroid glands. The exact prevalence of hypoparathyroidism in Wilson's disease is not known,though it seems to be a rare finding, the cause of hypoparathyroidism in Wilson's disease is deposition of copper in parathyroid gland. There is no report of hypoparathyroidism as the first manifestation of Wilson's disease in literature , hence this patient is most probably the first case of Wilson's disease presenting with hypoparathyroidism as the first manifestation.