A woman in her 50s presented to our otolaryngology clinic with a 3-month history of persistent nasal obstruction of and occasional epistaxis from the left side of her nose. She denied having symptoms associated with aural or cranial nerve involvement. However, a nontender, firm, 3 × 4-cm mass fixed over the upper left side of her neck was detected. The patient was a nonsmoker, and her medical history was not remarkable. The flexible nasopharyngoscopy revealed an unclearly delineated fleshy mass obliterated the left side of the posterior choana (Video 1 and Video 2). Axial T1-weighted gadolinium-enhanced fat-suppressed magnetic resonance image revealed an expansile tumor originating from the posterolateral recess of the nasopharynx, with direct extension into the left sinonasal cavity (Figure, A). The patient then underwent an immediate biopsy. The histopathologic findings revealed an image consisting of nests of small round to oval cells with scant cytoplasm, finely dispersed chromatin, nuclear molding, and inconspicuous nucleoli (Figure, B). Prominent mitotic activity and necrosis, cellular fragility with crushed artifact, and formation of pseudorosettes were readily identifiable. Use of immunohistochemical staining showed these cells to be positive for neural cell-adhesion molecule CD56 (Figure, C), with a dot-like staining pattern for pankeratin marker (clone: AE1/AE3) ( Figure, D), but negative for thyroid transcription factor-1 (TTF-1), synaptophysin, chromogranin A, neuron-specific enolase, cytokeratin 20, CD99, and leukocyte common antigen. These cells also showed strong cytoplasmic expression of p16 but were negative for human papillomavirus (HPV) by in situ hybridization. Subsequent positron emission tomographic-computed tomographic scanning revealed no evidence of distant metastasis.