Abstract: Primary brain calcification(PBC) is a hereditary neurodegenerative disorder with symmetrical calcification in the bilateral basal ganglia and other brain regions as its core imaging feature, and presents diverse heterogeneous clinical manifestations such as movement disorders, cognitive impairment, and psychiatric disturbances. The identification of multiple causative genes has not only facilitated the exploration of PBC pathogenesis but also continuously expanded its phenotypic spectrum. The diagnosis, evaluation, and management of this disease are experiencing pivotal shifts, accompanied by new challenges. In 2025, the first international expert consensus on PBC was published, laying a foundation for the standardized diagnosis and treatment of PBC worldwide. Based on China′s clinical practice and research status, and referring to the international consensus, domestic experts in related fields conducted multiple rounds of discussions, supplemented and revised the contents covering clinical manifestations, auxiliary examinations, diagnostic criteria, differential diagnosis, molecular classification and characteristics, genetic testing and counseling, disease management and treatment of PBC, and finally developed this consensus. This consensus aims to provide guidance for the standardized diagnosis, treatment, and research of PBC in China, help improve the quality of diagnosis and treatment, and drive the steady advancement of relevant research in this field.