Calcifying odontogenic cyst (COC) is a unique odontogenic lesion with combined features of a cyst and neoplasm. This lesion shows a great diversity in its clinicopathological behavior. It may become aggressive and rarely malignant. This case is an aggressive COC of the maxilla in a young male patient which recurred after several radical surgical excisions. Eventually invading the cranium, the lesion neither responded to surgical excision nor to radiotherapy.