In 1953 we1 first described the "Syndrome of Congenital Hemihypertrophy, Shortness of Stature, and Elevated Urinary Gonadotropins" in two children, a girl aged 5½ years and a boy 6 years old. Subsequently Silver and Gruskay2 reported a case of hemihypertrophy and elevated urinary gonadotropin in a 7-year-old boy with normal height. In none of these cases was there any other evidence of apparent endocrine disturbance. The purpose of this paper is to report two additional cases of this syndrome: the first in an 8½-year-old Negro boy with shortness of stature and elevated urinary gonadotropins in whom the difference in the two sides of the body was limited to the spine, and the other in an 8-month-old white boy with shortness of stature, a difference in size of the legs, and swelling of the dorsa of the hands and feet. A tabulation of the findings in these five cases