Background: Carpal Tunnel Syndrome (CTS) in the pediatric population is uncommon. The etiology and presentation is different from adults. There is debate in the literature about the treatment of CTS in children. Methods: We performed a retrospective review on all children (up to age 16 years) presenting with CTS from 1974 to 2005. Patient demographics, etiology, presenting signs and symptoms, treatment therapy, and long-term outcome were analyzed. Results: Thirty-two patients were diagnosed with CTS, either on history or nerve conduction tests (NCT) and electromyography (EMG). There were 21 females (66%) and 11 males (34%), with a mean age of 14 years (Range: 5 – 16 years). Twenty patients (63%) complained of bilateral symptoms. Overuse syndrome was the major cause of CTS (n=14, 44%). The signs and symptoms varied according to the etiology of the CTS. The success of the treatment modalities depended on the etiology of the CTS, with conservative therapy successful for overuse syndrome and surgery more successful in treating CTS secondary to genetic disorders and post-traumatic CTS. A treatment algorithm is presented. Conclusion: Overuse syndrome is becoming the leading cause of pediatric CTS. The presenting signs and symptoms as well as treatment modalities vary depending on etiology.