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Introduction: Chondrosarcomas belong to the most common malignomas of the skeletal system. The vast majority is located at the epiphysis of the long bones or in the pelvis. Less than 10% of chondrosarcomas are located in the head and neck area, most common in the bony viscerocranium and the larynx. Five histological variants can be differentiated: conventional, mesenchymal, myxoid, dedifferentiated and in less than 5% clear cell chondrosarcomas (CCCS). This case report gives the first description of a CCCS originating from the nasal septum. Case Report: A 79 year old female complaining about a 6 month history of nasal obstruction revealed a huge ballooning of the basal septum in anterior rhinoscopy. Computed tomography exhibited a septal tumor with amorphous calcification. Histology of the tumor resected via lateral rhinotomy confirmed a diagnosis of CCCS. Because of clear surgical margins no radiation therapy was performed. The patient stayed free of recurrence during follow-up of sixteen months. Discussion: The patient presented with typical symptoms of nasoseptal tumors as well as representative CT-scans and histologic findings of CCCS. Because of the complete resection and the CCCS-subtype, the patient is expected to have a positive prognosis.