<p>Endolymphatic sac tumors are rare neoplasms arising from the posterior aspect of the petrous temporal bone and are characterized by locally aggressive behavior with significant bone destruction. Although they may occur sporadically, a well-established association with von Hippel–Lindau (VHL) disease has been reported. This study aimed to characterize the clinical features, management, and long-term outcomes of a consecutive series of seven patients treated at a tertiary referral center, including cases associated with VHL. A retrospective analysis of institutional cases was performed and complemented by a narrative review of the literature. Three patients underwent primary surgical management and four were treated in a salvage setting after previous interventions elsewhere; three patients had genetically confirmed VHL disease. Gross total resection was achieved in one patient, subtotal resection in six, and adjuvant radiotherapy was administered in five. Clinical follow-up ranged from 9 to 28 years (mean, 13 years), with no radiological progression observed during post-treatment follow-up; at last evaluation, five patients were alive with stable residual disease, one had no evidence of disease after gross total resection, and one had died from an unrelated cause. Within the limitations of a small retrospective series, these findings suggest that maximal safe cytoreduction combined with individualized multimodal management may represent a reasonable strategy in selected advanced or previously treated cases.</p>