Lymph node enlargement is a common finding in children suggesting normal or benign. Palpable nodes which are large, hard or fixed must be examined carefully to rule out malignant diseases. In this case, a 15-year-old boy presented to our hospital to inspect the palpable mass at his post-auricular area being found 2 months ago. It was diagnosed as nodal marginal zone lymphoma (NMZL) through excisional biopsy and immunohistochemistry. NMZL is very rare, especially in children and young adults, but occurs locally in most cases with a good prognosis compared to adults. We described a rare case of NMZL diagnosed in adolescent.