Neurothekeomas are uncommon benign soft tissue tumors of nerve sheath origin. They occur predominately in the head and neck or upper trunk of children and young adults. A 15-month-old boy presented with an enlarging mass of the right maxilla. Radiologic imaging demonstrated an expansile lesion of the nasomalar region. An incisional biopsy resulted in the diagnosis of neurothekeoma. This lesion should be considered as part of the differential diagnosis of pediatric soft tissue head and neck masses. We discuss the presentation, evaluation, and treatment of these rare benign lesions.
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