Desmoid tumors are benign tumors that cause considerable morbidity and are prone to recurrence. They tend to extensively infiltrate surrounding tissues, complicating the treatment. We present the report of two cases of desmoid tumor in the pediatric population. The first patient had tumor that necessitated removal of most of the anterior compartment of his leg. The tumor in the second case was intimately involved with neurovascular structures and, therefore adjuvant treatment including chemotherapy and repeat surgery was necessary. We present these cases as well as a review of the literature to illustrate the challenges in diagnosing and treating pediatric desmoid tumors.