Aim: The importance of clinical history, meticulous clinical examination, perioperative monitoring, team work in diagnosing and treating rare diseases for a better patient outcome. Case report: 26 year old Gravida 2 at term was admitted with sudden onset of severe bifrontal headache, vomiting and diagnosed to have frontal lobe abscess with mass effect. Considering that she was at term, Lower segment caesarean section (LSCS) was done prior to emergency craniotomy for better fetal outcome. Discussion: Due to persistent perioperative hypoxemia, post operatively the possibilities of congenital heart disease and deep vein thrombosis were ruled out. Computerized Tomographic pulmonary angiogram (CTPA) revealed multiple AVMs in left upper lobe and an AVM in the anterior segment of left lower lobe. On reevaluation history and clinical findings clinched the clinical diagnosis of Osler-Weber- Rendu Syndrome (OWR) or Hereditary hemorrhagic telangiectasia (HHT). Fortnight later she underwent surgical excision of PAVMs. Conclusion: This case report highlights the importance of clinical history, meticulous clinical examination, perioperative monitoring and team work in diagnosing rare entities like PAVM, Hereditary hemorrhagic telangiectasia, in a term pregnancy with cerebral abscess.