Objectives: Oral cancer is the eighth most common type of cancer worldwide and a significant contributor to the global burden caused by this disease. The principal parameters considered to influence prognosis, and thus treatment selection, are size and location of the primary tumor, as well as assessment of the presence and extent of lymph node and distant metastasis (DM). However, no known report regarding the relationship between the primary site and DM has been presented. For effective treatment selection and good prognosis, the correlation of DM with anatomic site and histopathology results of the primary malignancy is important. In the present study, we performed a systematic review of published reports in an effort to determine the relationship between the anatomic site of various types of oral cavity cancer and DM. Methods: A systematic review of articles published until the end of 2018 was performed using PubMed/MEDLINE. Results: A total of 150 studies were selected for this review. The percentage of all cases reported with DM was 6.3%, ranging from 0.6% to 33.1% in the individual studies. The rate of incidence of tongue occurrence was 9.3%. A frequent DM site was the lungs, with adenoid cystic carcinoma the most commonly involved histopathological factor. Malignant melanoma was most frequent (43.4%) in all histopathology findings, whereas there were no cases with an acinic cell carcinoma or cystadenocarcinoma. Conclusions: We found that the occurrence of DM from the primary site as well as rate of incidence was dependent on histopathological factors.