Abrikossoff's tumor is a disease that more commonly affects the oral cavity but can also occur at other sites. It develops between the second and sixth decades of life, more frequently among women and blacks. The neoplasm can affect all parts of the body. The head and neck areas are affected in 45% to 65% of cases and of these, 70% are located interorally (tongue, oral mucosa, hard palate). The benign form shows polygonal cells with granular, eosinophilic cytoplasm and small nuclei. The malignant form, however, is associated with a high mitotic index and pleomorphic cellular tissue. The clinical aspect of the neoformation is a swelling covered by mucus of normal appearance. Studies of the neoformation show that in addition to the objective examination, further instrumental research is necessary, i.e., with nuclear magnetic resonance or computed tomography with contrast CT scan. However, the only examination that can confirm the clinical diagnosis is the histological examination. The only treatment for Abrikossoff tumor is surgery. The surgical treatment provides for an extirpation of the neoformation with the overhanging mucus and the underlying periosteum. In this work, the authors discuss a case of Abrikossoff tumor affecting the mucus of the right side of the hemipalate in a 53-year-old patient and present a review of the literature.