24-year-old woman at 28 weeks gestation was referred from peripheral hospital with diagnosis of pregnancy with portal hypertension. She had received multiple transfusion for pancytopaenia in the past and had undergone endoscopic sclerotherapy for oesophageal varices. Initially, she was admitted in our hospital at 28 weeks gestation for blood transfusion and was evaluated by multispecialty team of doctors. She was advised splenectomy for transfusion-dependent pancytopaenia secondary to hypersplenism in non-cirrhotic portal hypertension. She was readmitted at 36 weeks gestation. A decision for caesarean was taken owing to failed induction of labour at 38 weeks gestation. She underwent combined caesarean with splenectomy. Mother and child had an uneventful postoperative recovery and were discharged on ninth postoperative day. Preconceptional counselling, treatment of oesophageal varices and multispecialty approach was paramount in the management. Combined caesarean with splenectomy is feasible and cost-effective treatment associated with improved quality of life. Prospective clinical trials are essential to prove safety and efficacy of treatment.