Neurothekeoma is a benign soft tissue tumor commonly located on the skin. In this report, a 3-year-old boy presented with restricted right eye movement and decrease in visual acuity. The patient was found to have a 6-cm neurothekeoma involving the maxillary and ethmoid sinuses. To our knowledge, this is the first reported case of neurothekeoma with involvement of the maxillary and ethmoid sinuses. This uncommon lesion should be considered as the differential diagnosis of pediatric soft tissue tumors in the head and neck region.