Ameloblastic carcinoma is a kind of rare malignant tumor of jaws. The epidemiology, treament modality, and prognosis are not well-understood because there are few literatures of ameloblastic carcinoma reported up to date. In general, ameloblastic carcinoma is a highly aggressive tumor with rapid growth, and cases of distant metastasis had been reported. Surgical resection is the most common treatment modality, while there is still controversy between neck dissection, radiotherapy, and chemotherapy. A 25-year-old male, who had been treated for ameloblastoma of the maxilla two years ago, visited our hospital due to bleeding in the nasal and oral cavity for 2 months. Physical examination disclosed a nontender mass in the right maxillary mucobuccal fold and the magnetic resonance imaging revealed a solid mass occupied in the right maxillary sinus. Enucleation combined with peripheral ostectomy was performed. Histologic examination of the excised lesion showed ameloblastic carcinoma. Postoperative adjuvant radiotherapy was carried out. Healing was uneventful and following up showed no evidence of recurrence and distant metastasis.