To investigate the prevalence of malignant and potentially malignant lesions of the lip in an oral pathology service and to compare these data with a literature review. A total of 3173 biopsy reports and histopathological records were analyzed. Cases with a histological diagnosis of actinic cheilitis (AC) with or without epithelial dysplasia, in situ carcinoma, or lip squamous cell carcinoma (LSCC) were included. A comprehensive literature review was conducted to investigate the prevalence of AC and/or LSCC. 124 cases (3.91%) were included, 75 (60.5%) had some degree of epithelial dysplasia and 31 (25.0%) were LSCC. Clinically, most of the lesions were diagnosed as AC (50.8%); however, eight cases clinically reported as AC were histologically diagnosed as LSCC. Regarding clinical characteristics, most individuals were fair-skinned male, with mean age of 54.3±12.3 years, and with a history of long-term solar exposure. Furthermore, 18 articles were selected from the literature, showing that the lower lip was predominantly affected and that most individuals were males, fair-skinned, and older than 40 years. Since most of the cases diagnosed clinically as AC presented some degree of epithelial dysplasia, it is important to emphasize the value of biopsy and the histological evaluation of this lesion.