Low-grade thyroid-like nasopharyngeal papillary adenocarcinoma is a rare malignant tumor characterized by morphologic analogy to papillary thyroid carcinoma and aberrant thyroid transcription factor-1 (TTF-1) expression. To date, a limited number of cases have been reported in the literature. We report a case of a 20-year-old Chinese male patient with complaints of headaches and nosebleeds for 5 months. The histopathologic examination showed thyroid-like low-grade nasopharyngeal papillary adenocarcinoma that was confirmed by immunohistochemical staining. The patient was treated with macroscopic complete resection without adjuvant therapy. The epidemiologic characteristics and clinical and pathologic features of the disease are summarized. In addition, we summarize the treatments used previously. In general, TL-LGNPPA is a very rare low-grade adenocarcinoma with aberrant expression of TTF-1, and its diagnosis depends on morphology and immunophenotype. The prognosis is good.