Objective To report the first case of using assisted reproductive technologies (ART) for fertility preservation in a patient with mixed connective tissue disease (MCTD), secondary pulmonary hypertension (PH) and antiphospholipid syndrome (APS). Design Case-report and review of the literature. Setting Academic infertility practice and tertiary care center. Patient(s) A 25-year-old woman with MCTD, complicated with PH and APS, who was scheduled for gonadotoxic therapy Intervention(s) Controlled ovarian hyperstimulation, egg retrieval, embryo banking. Main outcome measure(s) Successful ART cycle leading to embryo banking without worsening her underlying disease. Result(s) Following successful embryo cryopreservation, the patient experienced respiratory failure and other severe complications, resulting in a prolonged hospital stay. Conclusion(s) Controlled ovarian hyperstimulation for embryo banking in women with MCTD, PH and APS may pose a risk for potentially catastrophic complications. A multidisciplinary approach to these patients is necessary to optimize the outcomes of such procedures. More data are needed regarding the safety of fertility preservation technologies in patients with complex medical diseases.