Endolymphatic sac tumor (ELST) is a rare, aggressive hypervascular tumor of the posterior petrous bone. These tumors can manifest independently or in conjunction with Von Hippel-Lindau (VHL) syndrome. Currently, the primary treatment strategy involves complete surgical excision, though the role of radiotherapy remains debated.We are reporting a case of a 35-year-old female patient affected by ELST associated with VHL syndrome, managed solely through stereotactic radiosurgery and monitored by time-resolved imaging of contrast kinetics magnetic resonance sequence.