Differentiated thyroid carcinoma is a rare malignancy in children. It represents 0.4% to 3.0% of all childhood malignancies, with greater than 70% of cases presenting between the ages of 11 to 17 years and is exceptionally rare in children under 5 years of age. The most common type of differentiated thyroid carcinoma in children is papillary thyroid carcinoma, most of which are believed to be related to radiation exposure and only approximately 5% of cases have a family history of papillary thyroid cancer. In this report, we present a papillary thyroid carcinoma in a 3-year-old American boy with a family history of thyroid cancer and no known history of radiation exposure. A literature review with discussion on the management and treatment of pediatric papillary thyroid carcinoma follows.