Protein C deficiency increases the risk of thrombosis due to the lack of anticoagulant factor protein C. Among the numerous congenital protein C deficiencies, homozygous protein C deficiency has an especially low protein C activity level, that it is almost undetectable. It is a rare disease with a probability of 1:250000~500000. The signs and symptoms of homozygous protein C deficiency include purpuric, necrotic dermatosis, ecchymosis, blindness, and thrombosis in central nervous system. A 4-year-old girl was brought to the clinic with a chief complaint of extensive caries. The child was under warfarin medication in order to prevent possible complications during dental treatment. We consulted the pediatric department. Without warfarin intake, serious complications may occur due to thrombosis during dental treatment. Therefore, certain warfarin dosage (INR 3~5) and fresh frozen plasma as a backup for excessive hemorrhage were recommended. This child was a severely disabled child with the loss of vision, and it was difficult to manage her behavior effectively. Thus, dental treatment was carried out under general anesthesia, where bleeding control would be also easier to achieve.This report presents the case of a 4-year-old girl with protein C deficiency, who has received dental treatment for extensive caries under general anesthesia.