Turner syndrome is a sex-chromosome disorder occurring in one out of 2500 female births and characterized by growth retardation , gonadal dysgenesis and cardiovascular anomalies. The 45, XO karyotype is the most frequent type of this disease. Herein, we report on a 6-year-old girl with Turner syndrome and 45, XO karyotype presenting with short stature. She had dextrocardia and hiatal hernia. To the best of our knowledge, the association of Turner syndrome, dextrocardia and hiatal hernia is quite rare. Iran J Med Sci 2005; 30(2): 97-99.