A 16-year-old male developed holocranial headache with projectile vomiting 3 years ago and subsequently developed sudden quadriparesis. Subsequent CECT revealed a posterior fossa fourth ventricle tumor with hydrocephalus. He underwent a suboccupalcraniatectomy with CI laminectomy with biopsy of the tumor along with VP shunt and then was referred to SGPGIMS. He underwent DSA embolization in view of hypervascular tumor and in preparation of excision. Subsequently, as his biopsy was suggestive of hemangioblastoma and he had a strong family history (his grandfather died of a brain tumor at the age of 45, his cousin brother was operated for a bilateral PCC and his paternal uncle was diagnosed with hemangioblastoma), he was suspected of VHL and CECT abdomen was done that revealed bilateral adrenal lesions and pancreatic and renal cysts; subsequently, elevated urinary MN/NMN (5,476 mcg/ml) confirmed bilateral PCC and he was taken up for bilateral adrenalectomy after adequate alpha blockade. HPE of adrenal lesion revealed bilateral tumors without hyperplasia. He was then transferred to neurosurgery unit where he underwent re-exploration and complete excision of brainstem hemangioblastoma. However, in postop period he gradually deteriorated and later developed septic shock and eventually succumbed.Genetic mutation revealed heterozygous VHL: C277G known pathogenic variant ({"type":"entrez-nucleotide","attrs":{"text":"NM_000551.3","term_id":"319655736","term_text":"NM_000551.3"}}NM_000551.3 [VHL]:c.277G>Cin exon 1. Protein change glycine to arginine [p. Gly93Arg]).