The purpose of this study was to report a rare case of an aggressive nasopalatine cyst with nasal involvement in an edentulous patient. A 61-year-old edentulous Caucasian male presented with an anterior maxillary lesion that prevented him from wearing his complete upper denture. Clinical examination showed vestibular and palatal swelling around the edentulous sites of teeth #11, 12 and 13. Radiological examination revealed a well-limited cyst of the anterior maxilla that was aggressive in nature because of its size, erosion of the palatal and buccal cortical bone, and extension to the nasal floor. The provisional diagnosis was an aggressive nasopalatine duct cyst, but because the patient had been edentulous for years, the differential diagnosis also included a residual cyst. To remove the lesion, we used a slightly modified surgical procedure compared with the conventional approach: a flap was raised on the palatal side and another on the vestibular side instead of the classical palatal approach. The diagnosis of a nasopalatine duct cyst was confirmed after a histopathological examination. Large destructive nasopalatine duct cysts involving the nasal cavity are rare. In an edentulous patient, this lesion may be misdiagnosed as a residual cyst. In this case, the surgical risk was the inadvertent creation of a naso-antral communication. The lesion was completely enucleated without complications and healing was uneventful.