Five SRS children, 21.7% of the group, (3 females and 2 males, 2 11p15LOM and 3 mUPD7) experienced central precocious puberty; 4 out of 5 were treated with gonadotropin-releasing hormone (GnRH) analog; 1 girl who came to our attention at the age of 15 years had experienced menarche at the age of 10 years while the two boys (1 with 11p15LOM; 1 with mUPD7) showed a biochemical central precocious pubertal activation but with small testis (testicular volume 3 ml in both subjects). Here, GNRH1 is linked to precocious puberty.