Insular-type carcinoma of the thyroid is a rare form of undifferentiated thyroid cancer. The manifestation of disease occurs mainly in adults and is extremely rare in children. Prognosis of this type of thyroid carcinoma is unfavorable in childhood. Because of its rarity, it is not yet clear whether these tumors carry the same risk of progression. Therapy of choice is total thyroidectomy combined with a cervical lymph node dissection. In children, surgery is associated with a higher risk of recurrent nerve palsy and loss of parathyroid glands than in adults. Therefore, identification and protection of the recurrent laryngeal nerve using electrical neuromonitoring as well as exact preparation of parathyroid glands may reduce these risks. The history of a 10-year-old girl with insular-type thyroid carcinoma is presented. Surgical considerations such as total thyroidectomy vs less-than-total thyroidectomy with unilateral or bilateral cervical lymph node dissection are presented. Prognosis concerning morbidity, pointing out the aspect of electrical neuromonitoring and precaution of parathyroid glands, and survival rate of this extremely rare entity are discussed.