Introduction: Osteosynthesis imperfecta (OI) is a rare genetic disease that affects the collagen genes COL1A1 and COL1A2, may also be caused by recessive mutations in other genes. Its clinical spectrum includes from lethal perinatal forms to milder forms, its diagnosis can be established clinically, before a patient with repeated fractures, blue scleras, short stature, dentition disorders and deafness.Case presentation: 24-year-old third-party patient with an obstetric history of a living child, a dead child (osteogenesis imperfecta) and the current pregnancy that comes to the institution’s office with results of chromosomatia, obstetric and structural ultrasound confirming product with osteogenesis imperfecta It is evaluated with strict regime by external consultation and termination of pregnancy is planned at 38.2 weeks of gestation by last date of menstruation plus pelvic plus previous caesarean section, newborn is received with multiple fractures in extremities and thorax so it is admitted to the service of neonatology where he dies within 24 hours due to pulmonary complication.Conclusions: it is important to diagnose early in the first trimester of pregnancy in order to establish a prognosis and timely treatment in case of meriting it.