Abstract Cases with maxillary cancer are most frequently found in the 5th to the 7th decades of life, and then rarely occur in adolescence, even less so in a children. One does not expect to encounter this tumor in children, considering that most have a non-epithelial origin. However, we had a case of maxillary squamous cell carcinoma which appeared in a 12-year-old boy. He was initially considered to have sinusitis, and received conservative treatment for a while. After diagnosis, a combination therapy consisting of preoperative irradiation along intra-arterial administration of 5-FU and CBDCA was followed by total maxillectomy and primary reconstruction. Unfortunately about 2 months later, distant metastasis developed along with local recurrence, and the boy died about 1 year 6 months after the initial presentation. The clinical details of this rare case will be discussed in conjunction with some important considerations in dealing with this type of malignancy in a child.