Abstract: Myoepithelial carcinoma is a rare malignant tumor. The primary site of this tumor is the salivary glands. With the rapid advancement in immunohistochemical examination and pathological examination, the number of clinical cases of the most common type of myoepithelial carcinoma of the salivary glands, as well as rare primary sites of myoepithelial carcinoma, reported in the literature has increased. Based on related cases reported in the literature, various studies worldwide, and our personal practical experience, this article summarized advancements in the research on myoepithelial carcinoma, including etiology, pathology, clinical characteristics, diagnosis, and treatments, as well as myoepithelial carcinoma features of various rare primary sites. This article aims to provide useful evidence for oncologists in diagnosing and treating myoepithelial carcinoma.