Head and neck tumours are among the top ten of all tumour entities. They make up a total of 30%, with 90% of these being squamous cell carcinomas. Upper jaw carcinoma is one of the rare sites in the mouth and is below 35%. The occurrence in childhood is extremely rare. Only a few publications are available. With 1800 childhood malignancies per year, the probability of occurrence is less than 0.2%. Boys are 20% more affected than girls. The prognosis quoad vitam of childhood-treated cancer cases under the age of 15 years at initial diagnosis is 81% over the next 15 years. The exclusion of typical childhood risk factors such as EBV-associated carcinoma or genetic disposition suggests a sporadic occurrence. We present a 13-year-old patient with an exophytic upper jaw carcinoma. Initially, neoplasia was histologically noted as a pseudoepithelial hyperplasia (PEH). After referring the sample to the Center for Dermatopathology and the Children's Cancer Registry, the diagnosis of carcinoma was revealed. Staging was performed, EBV and HPV were excluded as well as tumour predisposition syndrome. Complete tumour resection was performed, followed by reconstruction with Bichat's fat graft, palatal rotation-flap, and a Rehrmann-flap. No further therapy was performed. The young patient currently remains free of cancer after 3 years and is regularly seen at paediatric hematology and oncology departments and the oral and maxillofacial surgery. Quarterly clinical and MRI controls are performed. Bony reconstruction with free autologous iliace crest graft was performed at age 16, followed by implantation. Keywords: Oral cancer, child