Introduction: Caudal Regression Syndrome is a rare congenital anomaly characterized byvarying degrees of agenesis of the sacrum and lumbar spine, frequently associated with musculoskeletal, genitourinary , gastrointestinal, and cardiac malformations. Although the exact pathogenesis remains unclear, a strong association has been established between this condition and maternal pre-gestational diabetes mellitus. Case presentation: A female neonate was delivered at term via elective lower segment caesarean section with a birth weight of 3.52 kg to a 38-year-old mother with type 2 diabetes mellitus on insulin therapy . The mother had two previous uncomplicated pregnancies with healthy offspring. On examination, the neonate exhibited multiple congenital anomalies. There was shortening of the lower body segment with rhizomelic limb appearance. The lower limbs were positioned in a frog-leg posture with lower limb hypotonia. Bilateral overlapping toes and rocker-bottom feet were observed. The sacral dimple was absent. Plain radiography of the spine demonstrated absence of the sacrum. Spinal ultrasonography confirmed sacral agenesis, with the spinal cord terminating at a normal level and no evidence of meningomyelocele. Cardiovascular examination revealed a systolic murmur. Two-dimensional echocardiography identified complex cyanotic congenital heart disease with duct-dependent circulation. The parents were counselled regarding the guarded prognosis and the likelihood of staged palliative cardiac interventions. Multidisciplinary input was arranged for ongoing management. Conclusion: Maternal diabetes is associated with a significantly increased risk of major congenital anomalies and neonatal morbidity . Strict preconceptional and antenatal glycaemic control is essential to minimize such risks. Early recognition and coordinated multidisciplinary care are crucial to optimize outcomes.