Endolymphatic sac tumor is rare, locally aggressive hypervascular tumor of papillary structure, arising from the endolymphatic duct or sac in the posterior petrous bone. We present four cases with this tumor. Two patients were male and the other two were female. Age of each patient was 15, 52, 58, and 67 years. Three patients presented with progressive hearing loss and sustained vertigo for months to years and another one was referred for the tumor detected in routine medical check-up. Preoperative embolization was performed in 3 patients. Complete excision of the tumor was achieved in all patients using translabyrinthine or retrosigmoid approach. Herein, we describe the clinical and radiographic features, surgical treatment and pathologic findings with a review of the literature.