Objective: To summarize clinical features and our experience of the diagnosis and treatment of pediatric nasal neuroglial heterotopia (NGH). Methods: Clinical data of 13 nasal NGH patients in Beijing Children's Hospital from August 2014 to October 2019 were retrospectively reviewed, including 9 boys and 4 girls, aged from 1 to 38 months with median age of 5 months. Radiological workups and excision of nasal NGH under general anesthesia were performed for all patients. B ultra-sound and MRI were performed for all external and mixed lesions, while ultra-low-dose CT scan and MRI for all intranasal type. Surgical approaches were dependent on location and extent of the lesions according to radiographic workup, including extranasal or transnasal endoscopic approach. Patients were followed up regularly after operation to evaluate the effect. Initial presentation, locations, imaging findings, surgical approaches and follow-up results were analyzed through descriptive statistical method. Results: Eight nasal NGH patients presented with an internal nasal mass and nasal obstruction, which belonged to intranasal type. Three patients presented with an external nasal mass which belonged to extranasal type and 2 patients had mixed lesions. The sites included nasal dorsum (n=5), anterior to the middle turbinate (n=5) and olfactory cleft (n=3). Surgical resections were done through median rhinotomy approach (n=5) or transnasal endoscopic approach (n=8). All the operations were successful and no complication occurred. All cases were followed up from 3 to 65 months. No recurrence was encountered. Conclusions: Nasal NGH is a rare lesion with atypical clinical presentation. Preoperative imaging including CT scan and MRI is essential for evaluation of the location, extent of the disease and for making the surgical plan. Treatment requires complete surgical excision.