The pleomorphic adenoma is a benign tumor. The patient was 55 years old, with no particular pathological history. For 18 months, she had a progressive and permanent right nasal obstruction associated with intermittent epistaxis, rhinorrhea and chronic unilateral tearing. The nasofibroscopy showed a smooth reddish bleeding tumor of the right nasal cavity, reaching the lower part of the middle cornet. Oropharynx examination revealed on the right side a sessile and bulging tumor of the palate with respect of the mucosa, the tumor goes through the choanae into the oropharynx. The CT scan showed a large aggressive lesion process centered on the right nasal cavity with bone lysis of the posterolateral wall of the maxillary sinus and invasion of the infratemporal fossa. MRI showed a maxillary right naso-sinusal tumor process infiltrating the ethmoidal cells, the right infratemporal fossa with endobuccal extension and filling the nasopharyngeal lumen. The patient was operated through the right external para-latero-nasal, associated to an endonasal abord. Anatomo-pathological with immune-histochemical study was performed to ensure complete removal of the tumor and it showed a high grade cystic adenoid carcinoma.