Funding: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Background: Precocious puberty (PP) implies the appearance of physical and hormonal signs of pubertal development at an earlier age what is considered normal. The beginning of puberty at an earlier age can have serious impact on the psychosocial wellbeing of the growing children in addition to its economic burden on the family. PP can be classified as central or peripheral depending on its etiology and many syndromes were identified for this growth disorder. The diagnosis of PP still represents a challenge to physicians and multidisciplinary approach is a key step to find out the correct etiology and to decide about long-term therapy. Case Report: We report a 7 year-old girl with history of prematurity presented with right sided hemihypertrophy of the body and accelerated linear growth confirmed with clinical examination and growth chart. All possible relevant laboratory and radiological tests performed to assess of her clinical condition. The diagnosis of central precocity associated with Silver-Russell Syndrome was suspected based on the clinical ground. Conclusions: PP with hemihypertrophy should be investigated thoroughly to look for central or peripheral etiology. Despite identifying many etiologies, our patient is the first case to report with these unique features.