DISCUSSION: LAM have long been considered as contraindications to pregnancy because of a worse outcome. In the present case, the patient had a strong desire to have her baby. We conclude intense care, sufficient preparations before operation, proficient anesthesia, skilled nursing is necessary for the successful delivery of LAM patients. For pregnant women with LAM, a definite diagnosis is difficult according to ERS Guidelines. Unavailable radiological or invasive procedures may delay the exact diagnose. The optimal cut-off point for definite LAM diagnosis was 850.7 pg/ml and it was correlated with disease severity, presentations of chylous effusions as well as treatment responses to sirolimus . For now, sirolimus or everolimus, two mTOR inhibitors, appear to be the most important, and the efficacy of sirolimus in LAM treatment has been proved. However, some serious adverse events like peripheral oedema, pneumonia, cardiac failure and Pneumocystis jirovecii infection were happened when everolimus (2.5 mg/day escalated to 10 mg/day. We hope future research may cover a convenient diagnosis procedure, an effective risk/prognosis assessment as well as optimal management strategies for pregnant LAM patients.