Thyroid involvement in Langerhans Cell Histiocytosis (LCH) is rare. We report a 10-year-old Filipino male who presented with a rapidly enlarging goiter. Computed tomography scan showed thyroid and bilateral submandibular masses with malignant features, pulmonary blebs and hepatic cysts. Ultrasound-guided core needle biopsy findings were consistent with LCH and chemotherapy was initiated. This case demonstrates that LCH should be considered in patients with goiter. Multidisciplinary management is warranted to achieve proper diagnosis and institute timely treatment.