A case of valproic acid (VPA)-induced hepatic failure was reported. An 8.5-month-old female developed infantile spasm at 1 month of life. She was then treated with high-dose VPA and clonazepam (CNP). She manifested with frequent seizures in the presence of pneumonia. Gingival bleeding, as well as multiple punctuated hemorrhagic spots were observed on her face and cervico-thoracic areas. There was severe edema on her extremities, especially the lower extremities. A significant reduction of full blood count and severe hypoproteinemia were noted although transaminase ALT was still within normal range. Both serum VPA and CNP levels were beyond the reference range. Diagnosis of VPA-induced hepatic failure was based on the clinical and laboratory findings. This study reviewed the high risk factors, the pathogenesis, early diagnosis, prevention and treatment of VPA-induced hepatic failure based on the case report and published literatures.