Background: Congenital hypothyroidism is one of the most encountered endocrine diseases in childhood and the importance of this diagnosis is because early and adequate treatment prevents developmental retardation and other squeal of the condition.Objective: Looking for the congenital hyperthyroidism in Iraqi children. Methods: A retrospective study involved 40 children who were attended Pediatric Endocrine and diabetic Clinic for children in Al-Kadhimiya Teaching Hospital, College of Medicine, Al-Nahrain University Baghdad, Iraq, over period from Jan 1993-Jan 2003 .Results: Of the 40 patients, 24 were female and 16 male. Female to male ratio 1.5:1.A history of parental consanguinity was positive in 36(80%) of the patients and 25(62.5%) had family history of hypothyroidism, and 24(60%) of the patients were from urban area 16(40%) from rural area west to Baghdad. Ten children (25%) detected in first month of life, 15(37.5%) in first 3 months and 25(62.5%) within the first 6 months. Seven patients had aplasia of thyroid gland, five had ectopia,and 8 had thyroid in normal position . Developmental assessment performed on all patients, also clinical manifestation in 20 patients diagnosed before 6 months of life.Conclusion: Although we do not know much about the overall prevalence of congenital hypothyroidism in Iraq, the first observation to make is that it is not a rare disease in this country. A genetic explanation suggested by the involvement of multiple siblings and the high rate of consanguinity in this population.Key Words: Congenital hypothyroidism, Retrospective