Short stature is a very common cause of referral. There are many causes of growth failure, but the great majority of cases are due to normal variants and only in a minority of cases an underlying disease is found. Pathological growth failure includes the consideration not only of short stature, but also growth velocity, genetic height and estimation of adult height. Idiopathic short stature is a broad concept difficult to define and in the last years new genes related to the development of the growth cartilage have been implicated. The diagnostic process should be stepwise and aims to differentiate between short stature that is a variant of normal and pathological short stature, identify the cause, and initiate treatment as soon as possible. A complete medical history and examination, together with a careful assessment of auxology, bone age, and basic complementary tests, allow the diagnosis to be guided without the need for advanced studies. Treatment with recombinant human growth hormone (rhGH) should be carried out under the indicated conditions and doses and started as soon as possible during prepuberty. This review presents the state of the art regarding differential diagnosis and therapeutic possibilities.