Endolymphatic sac tumor (ELST) is a rare (1/30000) neoplasm with less than 200 cases reported. It is seen in 24–30% of patients with Von Hippel-Lindau (VHL) syndrome. We present a case of a woman diagnosed to have left cerebello pontine angle endolymphatic sac tumour with low grade adenocarcinoma, underwent left radical mastoidectomy and tumour decompression; developed left cerebellar vermian haemangioma and a left occipito temporal haemangioblastoma who successfully conceived and had an uneventful pregnancy and Caesarean delivery with good maternal and neonatal outcome, due to its rarity. High index of suspicion for an early diagnosis and a planned delivery in a tertiary care centre with a multidisciplinary approach are crucial for successful outcomes.