The emerging concept that aggressive adenomatous tumors of the temporal bone arise from the endolymphatic sac and constitute a distinct clinicopathologic entity merits wider recognition. These tumors share a common clinical pattern and exhibit consistent imaging and histopathologic features. Endolymphatic sac tumors (ELSTs) have been mistaken for other neoplasms such as paragangliomas, adenomatous tumors of mixed histology, ceruminomas, and choroid plexus papillomas. A review of the literature shows similarities among case studies of these aggressive adenomatous lesions. An analysis of the data supports the endolymphatic sac as an origin for these tumors. This report also presents an additional case of a less differentiated variant of this rare but important clinicopathologic entity.