Tyrosine hydroxylase deficiency induced dopa-responsive dystonia is a treatable neurometabolic disease,which is relatively rare in clinic. In this paper,we present a case of dopa-responsive dystonia caused by tyrosine hydroxylase deficiency and reviewed relevant literature to investigate the pathogenesis,clinical manifestations,diagnosis and treatment of dopa-responsive dystonia caused by tyrosine hydroxylase deficiency. Finally,we concluded that peripheral blood prolactin may be a biomarker of tyrosine hydroxylase deficiency,Genetic testing in patients with clinically suspected tyrosine hydroxylase deficiency may be the only way to confirm the diagnosis,and early identification of the diagnosis and levodopa treatment may significantly improve the prognosis.