Up now, several medications were proposed for the treatment of autoimmune hepatitis (AIH); however, because of the unclear pathophysiology of AIH, the most optimal treatment option needs to be elucidated. This systematic review sought to investigate the safety and efficacy of rituxiamb (RTX) in patients with AIH. A total of 27 studies were included in the present study. A total of 80 patients had the eligibility criteria, of which the majority of them were female (63 female and 17 male). Of the 80 patients, nine patients were pediatrics. The induction of remission and maintenance therapy were the most common indications for RTX in AIH. Of the 80 patients, we found complete remission in 55% of patients (n = 44) and partial remission in 11% of patients (n = 11). Of the nine pediatric patients, we found complete remission in 77% of patients (n = 7) and partial remission in 22% of patients (n = 2). Unclear response was also reported in 31% of patients (n = 25), which included four studies. 375 mg/m2 × 4 followed by 1000 mg × 2 was the most commonly applied RTX dosage used for treatment of AIH. RTX therapy was associated with infectious complications in six patients; however, one episode of cancer, death, mild conjunctivitis, and large bowel perforation were also reported. RTX is an anti-CD20 mAb and was shown to be effective for the treatment of AIH, but there is no consensus regarding the therapeutic role of RTX in AIH.