Abstract
Pediatric chordomas are rarely described in the literature with most cases being managed surgically followed by adjuvant radiotherapy for local control. We present a case of an 18-year-old female with thoracic chordoma causing significant mass effect resulting in tracheal deviation, esophageal compression, and splaying of the great vessels. Ultrasound-guided anterior left transcervical percutaneous biopsy of the neck with surgical pathology immunohistochemistry confirmed the presence of chordoma. The patient underwent extensive palliative debulking followed by radiation therapy leading to clinical improvement. This case demonstrated that an ultrasound-guided percutaneous biopsy is an essential procedure in the diagnosis and treatment of chordoma, which led to successful treatment when followed by surgery and radiation.