Tehran University of Medical Sciences Frontiers in Dentistry 2676-296X 22 0 2025 10 14 5009 5009 Parmiss Omidsalar Department of Oral & Maxillofacial Medicine, School of Dentistry, Bushehr University of Medical Sciences, Bushehr, Iran Monir Moradzadeh Khiavi Department of Oral & Maxillofacial Pathology, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran Nafiseh Sheykhbahaei Dental Research Center, Dentistry Research Institute, Tehran University of Medical Sciences, Tehran, Iran 4. Department of Oral & Maxillofacial Medicine, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran 2023 10 29 2023 12 18 Myelodysplastic syndrome (MDS) is a bone marrow clonal stem cell disorder characterized by the inability of immature blood cells to mature. Oral squamous cell carcinoma (OSCC) has not been previously reported in MDS patients without underlying diseases. We present a case of poorly differentiated OSCC in a 27-year-old patient with MDS. A literature review revealed 11 case reports about solid tumors in different organs of MDS patients. Among these, 5 articles reported head and neck carcinoma but none of them reported oral cancer in MDS patients. MDS predisposes patients to hematopoietic and non-hematopoietic malignancies. The oral cavity is one of the critical sites that needs to be examined periodically and regularly in MDS patients to detect OSCC in its early stages. https://fid.tums.ac.ir/index.php/fid/article/view/5009 https://fid.tums.ac.ir/index.php/fid/article/download/5009/2168