Aim: To discuss a case of papillary thyroid cancer with desmoid-type fibromatosis (PTC-DTF) highlighting the clinical presentation and management of PTC with DTF nodal metastasis. Background: Papillary thyroid cancer with desmoid-type fibromatosis (PTC-DTF) is a rare biphasic variant of PTC with an incidence of up to 0.5%. It is made up of two distinct components—the PTC component (classical epithelial carcinoma) and the DTF component (mesenchymal proliferation of spindled cells resembling fibromatosis). We report the youngest case of PTC-DTF and one of only five cases with nodal metastasis of the DTF component. Case description: A 17-year-old female presented with a 4 cm left-sided neck lump without cervical lymphadenopathy. Ultrasonography (US) and fine needle aspiration (FNA) showed thyroid imaging reporting and data system 4 and Bethesda 5 nodules. She underwent a total thyroidectomy with bilateral central lymph node dissection. Histopathology demonstrated a 45 × 40 × 35 mm tumor with extrathyroidal extension into strap muscle and lymphovascular invasion. There were three metastases out of four excised lymph nodes, with both PTC and DTF components. The epithelial component was positive for BRAF and the mesenchymal component was positive for smooth muscle actin (SMA) and catenin beta 1 (CTNNB1). The patient underwent adjuvant radioactive iodine (RAI) treatment. Conclusion: Diagnosis of PTC-DTF is challenging, as preoperative workup including examination and US does not differentiate for this rare subtype. Further, FNA can sometimes misdiagnose an aspirated DTF component as benign fibroma or schwannoma, so caution is advised in the context of clinically enlarging lesions. Management of PTC-DTF is difficult as it is a more aggressive form of PTC, with nodal metastasis of the DTF component adding to the complexity of management. Complete surgical resection is important, as the DTF component is not sensitive to RAI. These cases should be discussed at a multidisciplinary meeting. Clinical significance: Patients with PTC-DTF are at a higher risk of invasion and nodal metastasis. Surgical clearance is of utmost importance in the management of PTC-DTF. Multidisciplinary meeting (MDM) discussion and ongoing surveillance are prudent in the management of this rare condition.