Abstract: In 1930 Ullrich and in 1938 Turner described females with a syndrome of short stature, sexual infantilism and a pattern of characteristic minor anomalies including pterygium colli. This syndrome named initially as the Urlich-Turner syndrome, was later called Noonan syndrome. It was later shown that the Ullrich-Turner Syndrome (UTS) is caused by monosomy X or a structural abnormality of the second X-chromosome. Presented here is a case of Noonan Syndrome in a four and half year old boy.