Sacral agenesis, known also as caudal regression syndrome, is a rare congenital abnormality characterized by absence of a variable amount of sacrum, lumbar spine and associated neural elements. It is mostly seen in infants of diabetic mothers. Nearly all patients have genitourinary anomalies, neurogenic bladder being the most common. Other associated anomalies include cardiac, gastrointestinal and orthopedic. Here, we present a rare type of lumbosacral agenesis in an infant of a diabetic mother. It was diagnosed antenatally at 26 weeks of gestation.