Gonadotropin dependent precocious puberty in a boy with Prader Willi Syndrome

Prader Willi Syndrome is a severe obesity syndrome due to the absence of paternal expression of imprinted genes localized at 15q11.2-q13. It is characterized by short stature, hypotonia, and feeding difficulties in infancy. Hypothalamic pituitary dysfunction is seen in this syndrome resulting in growth hormone deficiency and hypogonadism. Thus, the usual manifestation of this syndrome will be delayed puberty and infertility. Gonadotropin-dependent precocious puberty is a very rare manifestation, where some cases are associated with growth hormone therapy. We report eight years and eight-month-old boy presented to us with Prader Willi Syndrome and type 2 diabetes, who developed central precocious puberty.