Objectives: Ghost cell odontogenic carcinoma is a rare condition characterized by ameloblastic-like islands of epithelial cells with aberrant keratinitation in the form of Ghost cell with varying amounts of dysplastic dentina. Material and Methods: We report a case of a 70 year-old woman with a rapid onset of painful swelling right maxillary tumor. Magnetic resonance showed a huge tumor dependent on the right half of the right hard palate with invasion of the pterygoid process and focally to the second branch of the trigeminal. Radiological stage was T4N0. The patient underwent a right subtotal maxillectomy with clear margins. Adjuvant radiotherapy was given. The patient was free of residual or recurrent disease 12 months after surgery. Results: The tumor was 3,9cm in diameter. It was spongy and whitish gray. Microscopically the tumor was arranged in nets and trabeculae, occasionally forming palisade. Tumoral cells had clear cytoplasm with vesicular nuclei. There was atipia and mitosi with vascular and perineural invasion. The excised tumor was diagnosed as a GCOC. Conclusions: Ghost cell carcinoma is a rare odontogenic carcinoma. Its course is unpredictable, ranging from locally invasive tumors of slow growth to highly aggressive and infiltrative ones. Wide surgical excision with clean margins is the treatment of choice although its combination with postoperative radiation therapy, with or without chemotherapy, remains controversial.