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Background: Pulmonary hypertension classified under WHO risk group IV in pregnancy poses serious maternal and fetal threat,with termination recommended before 22 weeks. Recently, more women with this condition choose to continue pregnancies, necessitating optimization of cardiovascular health for safe labour and delivery.This case exemplifies the effective outcome achieved through multidisciplinary efforts in managing a patient with pulmonary hypertension and coexisting lung pathology. Case details and management: A 28-year-old primigravida first presented to us at 25 weeks and 2 days of pregnancy with progressive dyspnoea (NYHA II to IV) and intermittent palpitations past 3 months and was admitted. Examination revealed tachycardia, tachypnoea, low oxygen saturation, pallor, clubbing, raised JVP, loud P2, and absent breath sounds in specific lung regions, with diffuse fine crepitations bilaterally. Investigations showed iron deficiency anaemia, with extensive bronchiectasis, right lung collapse along with minimal pleural effusion on chest Xray, and cardiac abnormalities, including raised pulmonary arterial pressure.Tests for infections and autoimmune conditions were negative, while bronchoscopy revealed distorted bronchial anatomy. Treatment included oxygen support, IV antibiotics, diuretics, anticoagulants, beta-blockers, and pulmonary hypertension therapy (PDE5 inhibitors). Elective preterm LSCS was performed at 32 weeks due to deteriorating maternal condition, delivering a live male baby (1.3 kg) who was shifted to NICU. The mother continued on HFNC, vasopressors, and epidural analgesia post-operatively. Both were discharged on postpartum day 34; the mother on nasal oxygen at 1-2 LPM, and she was off oxygen 3 months postpartum. Discussion: Pulmonary arterial hypertension carries a high risk of maternal mortality. Pre-conceptional counselling is of paramount importance for optimising cardiovascular reserve of patient wanting to continue pregnancy and early termination and contraceptive advice for patients wanting to avoid an unwanted pregnancy. Tailored multidisciplinary management is crucial for favourable outcome for mother and baby.