Benign smooth muscle tumors rarely occur in the head and neck and, to the best of our knowledge, have not been reported in the pterygopalatine fossa. In this report, we describe a 15-year-old adolescent who presented with facial pain and was found to have a large skull base tumor centered in the pterygopalatine fossa. The patient underwent an expanded endonasal endoscopic approach for complete resection of this lesion with resolution of his symptoms. Pathology revealed a well-differentiated smooth muscle neoplasm consistent with a leiomyoma. This case adds to the growing body of literature supporting a role for endoscopic procedures in the treatment of skull base pathologies in pediatric patients.