AbstractWe herein report the rare case of a 4‐year–5‐month‐old boy who presented with primary renal neuroblastoma. The patient developed repeated lung and liver metastatic recurrences, but, following a combination of chemotherapy, radiation therapy and aggressive surgical resection, the patient is now in remission. To investigate the pathogenesis of lung metastasis, immunohistochemistry was performed for matrix metalloproteinase‐9 and ‐14 (MMP‐9 and MMP‐14), molecular markers of invasion, metastasis and angiogenesis in neuroblastoma. In the present case, MMP‐9 expression was not observed, but MMP‐14 expression was detected in the primary lesion and was more highly expressed in the metastatic lesion compared with the primary one. Given the MMP‐14 staining in other cases, expression of MMP‐14 may be associated with the aggressiveness of the tumor. This suggests that selected clones with high MMP‐14 expression in the primary tumor might metastasize and form MMP‐14‐rich lesions.