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Paediatric thyroid cancer is a rare disease, but its incidence is rising in recent reports. This review aims at integrating recent findings into the current optimal diagnostic and therapeutic approach.The causal relationship of differentiated thyroid cancer (DTC) to radiation exposure is increasingly unravelled. Research progressively uncovers the genetic basis, such as RET (rearranged during transfection)/papillary thyroid cancer (PTC) rearrangement and RET-mutations. Knowledge of oncogenic signalling pathways nowadays starts to help finetuning diagnosis, prognosis and treatment. This knowledge complements the current state-of-the-art of paediatric thyroid cancer treatment. In childhood, DTC presents at a more advanced stage and implies higher recurrence rates, recurrences often occurring decades later. Treatment should minimize not only these recurrences but also long-term treatment sequelae. Total thyroidectomy and central compartment dissection by a high-volume surgeon and radioactive iodine is the preferred approach for most children with DTC. For children with medullary thyroid cancer within the MEN2 framework, when possible, prophylactic thyroidectomy is performed. Unfortunately, frequently, the diagnosis is still made at a later stage, and then requires total thyroidectomy with dissection of the central compartment and the lateral neck, when involved.The management complexity, the essential long-term follow-up and the lifetime burden of eventual complications demands management of paediatric thyroid cancer by physicians with the highest expertise. In such hands, excellent results can be obtained.