Introduction: Down syndrome (DS) is the commonest chromosomal abnormality and has multisystem involvement, including the anomalies of the kidney and urinary tract with a prevalence ranging from 3–20%. Data from South Asia remains limited. Objectives: To describe the prevalence, spectrum, clinical presentation, and renal complications of anomalies of kidney and urinary tract in children with DS. Methods: A descriptive cross-sectional study was conducted among children aged