The most common malignant lesion in the nasopharynx is the nasopharyngeal carcinoma (NPC), commonly presents with painless upper cervical lymphadenopathy secondary to nodal metastasis and the undifferentiated type is the most common diagnosis. Compared with other types of cancers, NPC is uncommon, albeit with a high difference in geographical distribution where the worldwide cases account for only 0.6% of all annually diagnosed cancers diagnosed, however, in Saudi Arabia the overall incidence among all age groups is 0.1–0.13 per 10 000. Other rare types of malignancies are also seen at the nasopharynx and due to the nonspecific clinical and imaging characteristics; the preoperative diagnosis may be difficult. This study retrospectively assessed the nasopharyngeal tumors diagnosed in a referral hospital in Al-Baha regions, Saudi Arabia during 10 years period that came with unusual presentations at the level of clinical, radiological, or pathological features to raise the awareness of healthcare provides by these entities. Twenty patients were included and revealed unusual (atypical) clinical presentation in 50% of case including a case presented with respiratory symptoms as a first presentation where multiple lung foci were seen and proved to be a metastatic NPC. Histopathological diagnoses were challenging for 40% of the cases revealing unusual malignant types. In summary, however, the nasopharyngeal malignancy is a rare entity, accurate diagnosis may be missed in clinical or even in the pathology examination. Surgeons and pathologists should be aware of the unusual presentation to provide early and more accurate diagnosis for such serous tumors.