Background: The specialists should identify the features of Wilson disease and autoimmune hepatitis when both affect a patient to adopt appropriate treatment. Objectives: This study was conducted to determine features of the patient, disease, diagnostic studies, and therapeutic measures in cases of simultaneity of Wilson disease and autoimmune hepatitis. Methods: To find evidence related to the study objectives, we searched databases such as Barakat knowledge network system, SID, Magiran, Google Scholar, Web of Science, ProQuest, Springer, ScienceDirect, Medline via PubMed, and Scopus with specified Persian and English keywords, including “Wilson’s Disease”, “Autoimmune”, and “Hepatitis”. The inclusion criteria for the studies were 1) the study was observational and 2) the study was published in Persian or English. The exclusion criteria included low-quality studies based on the score obtained from the checklist. The obtained studies were screened in terms of titles, abstracts, and full text, and finally, the qualified studies entered the review process. The relevant data were extracted according to a designed checklist. Results: Finally, 10 studies were included in the review process. Information about 14 patients was reported. The Mean±SD age of the participants in the studies was 19±11 years. The direction of diagnosis was from autoimmune hepatitis to Wilson disease in 8 cases and from Wilson disease to autoimmune hepatitis in 3 cases. The simultaneity of autoimmune hepatitis and Wilson disease was considered in 3 patients with no primary and secondary diagnosis. Conclusions: The comorbidity of Wilson disease and autoimmune hepatitis is uncommon but is important. In the presence of relevant symptoms in these patients, the comorbidity of these two diseases should be considered. Accordingly, additional assessments such as serum ceruloplasmin, urinary 24-h copper, molecular genetic testing, MRI, serological tests, anti-nuclear antibody, anti-mitochondrial antibody, anti-smooth muscle antibody, complement level, gamma globulin, IgG, albumin, Kayser-Fleischer ring eye examination, and liver biopsy should be considered for correct diagnosis. If appropriate treatment was started for the disease with a diagnosis of Wilson disease or autoimmune hepatitis, but the response to treatment was insufficient, it is better to consider the simultaneous occurrence of two diseases or the initial misdiagnosis.