Chondrosarcoma arising in the head and neck and craniofacial region is an uncommon lesion. The nasal septum is a particularly rare site of origin, with approximately 30 cases previously reported in the English literature. We present six new cases of chondrosarcoma arising in the nasal septum. Each of these tumors required cranial base surgical approaches for removal. Current imaging techniques allow a very accurate diagnosis to be made before biopsy. The characteristic ring-forming calcifications seen on computed tomography scans can be correlated with the histologic pattern of calcification. Magnetic resonance imaging techniques allow precise definition of tumor extent, which is particularly important because the disease is best treated with primary surgery. Advances in imaging and surgical techniques allow a much more complete tumor removal. It is hoped that this will increase the likelihood of cure in these patients. Surgical management and indications for adjuvant therapy are discussed.

Alternative approaches to the SMG are feasible but should be tailored to the individual patient based on factors such as pathology, patient preferences, availability of technology, and the experience and skill of the surgeon.