Abstract

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• We report on a surviving four-year-old female with sirenomelia. She was born with the features of fused lower extremities, imperforate anus, colon atresia, pelvic and sacral dysplasia. Despite these severe anomalies, she had functional renal units. Maldevelopment of the genitourinary tract in patients with severe variant of caudal regression syndrome is usually incompatible with life. The case report includes the most current imaging studies of her unusual pelvic anomalies which poses a reconstructive challenge to the pediatric surgeon.