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Sacral agenesis (part of the caudal regression syndrome) is a rare and severe sacral developmental abnormality. It is a congenital malformation of unknown aetiology with possible involvement of genetic and teratogenic factors. It is described by various degrees of developmental failure, the most extreme and rare being sirenomelia or mermaid syndrome. The associated malformations comprise anorectal, vertebral, urological, genital, and lower limb anomalies. Approximately 15-20% mothers of these children have insulin dependent diabetes mellitus. The case is being reported for its rarity and educative value because prognosis is good in isolated sacral agenesis.