Tethering of the spinal cord is frequently associated with myelomeningocele. However, orthopaedic surgeons must be familiar with the primary tethered cord, which is associated not with an overt myelomeningocele but with more subtle clinical findings, particularly in the pediatric patient. The charts of patients with tethered spinal cords from the Honolulu Unit of the Shriners Hospital for Crippled Children are reviewed to highlight potential diagnostic and treatment problems. Tethered spinal cord should be considered in the differential diagnosis of patients who present with isolated lower extremity deformities and/or subtle neurologic findings. Appropriate evaluation may require magnetic resonance imaging, ultrasonography, or metrizamide computed tomography. Urologic function must also be evaluated and monitored. The literature supports an aggressive diagnostic and, where indicated, surgical approach to the tethered spinal cord. Diagnosis and treatment at a young age has been shown to correlate with optimal results.