Three cases of Brachmann-de Lange syndrome with clinical, laboratory, X-ray and EEG examinations are reported. In one of the cases (case 1) a right scrotal hernia was observed. In the second one, four fingers in the right hand were noticed being the ring finger absent. Besides this, the X-ray revealed hypoplasia and subluxation of both radio bones. This child didn't cry, she screamed. Her karyotype was normal. The third case presented bilateral clinodactyly and microcephalia; her karyotype was also normal. A review of the medical literature led the AA. to diagnose their cases as Brachmann-de Lange syndroms whose etiology remains obscure.