Connective tissue diseases are rare usually affecting women of reproductive age. Patients should be informed about the potential obstetrical risks associated with their disease as well as potential exacerbations during pregnancy, while simultaneously being reassured with the prospect of a positive pregnancy outcome. In recent years, significant progress has been made in medical treatments, giving women the opportunity to consider pregnancy. Preconception counseling is critical to pregnancy planning. An effective contraceptive measure should be recommended based on disease activity and teratogenic medications should be modified. Pregnancy monitoring are managed based on specific clinical and serological markers (such as the presence of anti-SSA/SSB or anti-phospholipid antibodies). A multidisciplinary approach is essential for a safe pregnancy.