Objective: To assess the prevalence of renal and urological anomalies in patients with primary congenital hypothyroidism (PCH).Method: 202 patients with PCH recruited from DEMPU unit diagnosed both by ultrasonography and/or scintigraphy( 40.6% Dysgenesis, 59.4% Dyshormonogenesis). The study is both pro and retrospective study, in sixth month duration from Dec 2009- May 2010. Results: Prevalence of renal anomalies (4%), urologic anomalies (0.5%) and other anomalies (1.5%). There was no significant difference in the longitudinal follow up of growth and sexual maturation between hypothyroid with anomalies and those without. Statistical significant difference in Replacement therapy of both groups. Conclusion: Renal ultrasonography should be done to screen this birth defect as soon as possible so to prevent or delay the risk of ending in ESRD.