Abstract: ObjectiveTo explore the diagnosis and surgical treatment of congenital intranasal meningoencephalocele with concurrent ectorhinal glioma. MethodsClinical data of one child suffering from intranasal meningoencephalocele with concurrent ectorhinal glioma surgically treated in our department were collected and analyzed in combination with literature review. Imageological examinations including sinus CT scanning and cranial MRI were given to the child and surgical program was made according to the results. Under general anesthesia, the child received intranasal tumor resection and anterior skull defect repair endoscopically combined with ectorhinal tumor resection via an extranasal approach. Bloodbrain barrierpermeable antibiotic was used postoperatively to prevent infection. ResultsThe masses located in both the nasal root and nasal cavity were removed simultaneously, and the child got cured without complications such as cerebrospinal fluid leakage, meningitis, septum perforation, visual impairment and epilepsy. Oneyear postoperative followup showed no tumor recurrence. The postoperative pathology revealed glial cells in masses from both nasal root and nasal cavity, which confirmed the diagnoses of heterotopic glioma and meningoencephalocele. ConclusionSinus CT and enhanced cranial MRI are important for the diagnosis of congenital nasal meningoencephalocele in infants. Proper surgical approach should be adopted according to the position of the neoplasm. Endoscopic neoplasm resection has the advantages of clear vision, small damage, less bleeding and fewer complications. The key to repair the skull base defect is the choice of suitable materials, such as muscle, fascia, cartilage flap or bone sheet, to fill the leak according to the defect size.