Objective: This study aimed to discuss the pathogenesis, clinical manifestation, diagnosis, and treatment of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Case Report: The diagnosis and treatment of 2 cases with teratoma-associated anti-NMDAR encephalitis were summarized and the clinical data of patients reported by domestic and international studies were reviewed in this study. The 2 cases were both adolescent females who showed mental abnormalities as their main clinical manifestation. The patients were positive for anti-NMDAR antibody in their serum and cerebrospinal fluid, and gynecologic ultrasound detected ovarian teratoma. After diagnosis, the patients underwent teratoma resection, followed by pulse therapy of hormones and gamma globulin. Chemotherapy was performed to prevent tumor recurrence, and patients were in a stable condition. Conclusions: Teratoma-associated anti-NMDAR encephalitis is commonly seen in young women. The clinical manifestation of this disease is nonspecific, and the patients mainly have fever, psychosis, and seizure. Tumor resection and immune therapy are effective treatment strategies, and standardized chemotherapy should also be performed to prevent recurrence.