Neuroblastoma is the most common extracranial solid tumors of childhood. The most frequent anatomic site of the primary tumor is abdomen. Metastatic spread of neuroblastoma to lymph node, bone, bone marrow and liver are common at presentation. However, primary or metastatic involvement of sphenoid sinus is extremely rare. Here, we report a case with stage IV neuroblastoma presents with the involvement of the sphenoid sinus. To our knowledge this is the fourth case of neuroblastoma involved sphenoid sinus in English literature. (C) 2003 Elsevier Ireland Ltd. All rights reserved.