Nasopharyngeal carcinoma encompasses all epithelial tumors of epidermoid lineage that arise in this anatomic site. Two different histoclinical entities, squamous cell carcinoma (World Health Organization type 1) and undifferentiated carcinoma of the nasopharyngeal type (undifferentiated carcinoma of the nasopharyngeal type; World Health Organization types 2 to 3), share the primary site origin; the latter is more prevalent worldwide. Undifferentiated carcinoma of the nasopharyngeal type is a specific entity related to the Epstein-Barr virus with a particular geographic distribution among well-defined ethnic populations. Recent advances in the identification of the Epstein-Barr Virus genome, serologic markers, and environmental factors add to the knowledge and diagnosis of this disease. Improvements in irradiation techniques and modern imaging have increased local control, but distant failures remain the major problem in patients with locoregionally bulky disease, which is the most common form at presentation. The need for a staging consensus is being led by several new proposals incorporating computed tomography imaging. Neoadjuvant or simultaneous cisplatin-based chemotherapy combined with radiotherapy results in high local control rates and a high disease-free survival rate in several phase II trials. Ongoing controlled trials will soon establish a role for this multimodality approach. Metastatic or recurrent disease shows a high percentage of objective response rates and a substantial proportion of durable complete responses. This review focuses on recent advances in undifferentiated carcinoma of the nasopharyngeal type biology and management.