Nasopharyngeal carcinoma (NPC) is a non-lymphomatous, squamous cell carcinoma that occurs in the epithelial lining of the nasopharynx, an area that shows varying degrees of differentiation. Although relatively rare worldwide, NPC has substantial incidence and mortality in populations in Southeast Asia and in people with Southern Chinese ancestry. In Malaysia, NPC is a leading cancer type. In the clinic, NPC presents on a very wide spectrum. Therefore, a high degree of suspicion on the part of the clinician and an increased awareness by the patient is essential for the recognition of an early lesion. Early detection of the cancer is important as it affects the patient’s prognosis and the mode of treatment. Managing patients with NPC is very challenging as patients usually present late when the cancer is already in an advanced stage. Here, we review the challenges in the management of NPC.