Backround data: CRS also referred to as caudal dysplasia andsacral agenesis syndrome is a rare congenital abnormalityin which a segment of the spine and spinal cord fails todevelop. The etiology is thought to be related to maternaldiabetes, genetic predisposition, teratogens and vascularhypoperfusion, but not true causative factor has beendetermined. Since there is not a true cure, treatment isdiff icult, multidisciplinary, and largely supportive. Lowerlimb deformities with sensory or motor loss and neurogenicbladder call for intensive and long-term attention. Objective: To evaluate using the Magnetic Resonance Imaging(MRI) results, radiological and clinical data of pediatric patientswith spinal problems related to congenital or neuromuscularconditions using the MRI results. Study design: We presented 10 cases of caudal regressionsyndrome (CRS) among the 65 consecutive pediatric cases thatwere diagnosed with congenital spinal column deformitiesthat came to Department of Orthopedics and Traumatology,Baltalimani Bone Disease Hospital, İstanbul, Turkey between2006 and 2009. Material Methods: Clinical and radiological findings werereviewed to classify each patient to Renshaw s and Guille sclassifications. The mean age was 81 (30-180) months. Clinicaland radiological finding of these cases along with life timemanagement were outlined and literature were reviewed. Results: In this study 11.3 % of the patients were diagnosedwith CRS in combination with congenital and spina bifida (SB)related spinal deformities. This indicates that the conditionmay not be as rare as most studies suggest. Our retrospectivestudy allowed us to see the various concomitant conditionswhich often occur with CRS. Conclussıon: The goal of this study was to show in whichvariations may CRS patients come into the picture and toprovide insight into the CRS and management of this disorder. Caudal Regression Syndrome remains associated withstructural and systematic problems including genitourinary,gastrointestinal, orthopedic, neurological, respiratory andcardiac anomalies.First step of the early management ofCRS should be an accurate prenatal diagnosis. We needlarger studies to determine the utilities of the classificationsand to improve them. It is possible that there are under- ormisdiagnosed patients in many centers who are undertreatment for accompanying disorders. Key words: Caudal regression syndrome, congenital scoliosis,magnetic resonance imaging, sacral agenesis, sacral displasi,spina bifida.