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Backgound: Caudal regression syndrome (CRS) is an uncommon disorder in which there is abnormal development of the caudal end of the foetal spine along with many associated anomalies. Manifestations of this syndrome can vary from the absence of a few terminal coccygeal segments to complete lumbosacral agenesis. Its overall incidence is 1 in 60000 live births. It is more commonly seen in infants of diabetic mothers, with a 200-fold increased risk as compared to the general population. Case Report: We present a case of CRS in a foetus of a non-diabetic mother and discuss the role of radiology in early diagnosis and timely management. We also correlate initial abnormalities of the nervous system found on imaging with postnatal clinical deficits. Conclusions: This case highlights the rarity caudal regression syndrome in a foetus of a non-diabetic mother. Early diagnosis of CRS is important for appropriate management.