Silver-Russell syndrome is an inherited disease that is characterized by intrauterine and postnatal growth retardation, relative macrocephaly, triangular face, body asymmetry and clinodactyly of the 5th finger, Hypomethylation of the imprinting control region that is localized in chromosomal region 11p15 is responsible for 35-65% of the cases. We present here a case that has phenotypic characteristics of Silver-Russell syndrome and growth hormone deficiency, and a definitive diagnosis made with determination of imprinting control region hypomethylation.