We present a case of papillary cystic low-grade adenocarcinoma of endolymphatic sac origin. These tumors are very rare and only a few cases have been reported in literature. They have a protracted clinical course causing progressive invasion and bony destruction. Radiologically, they appear as a heterogenous lobulated vascular mass, invading bone and compressing surrounding structures. Histologically, these tumors are composed of uniform population of cells, resembling normal endolymphatic sac epithelium. This causes considerable diagnostic difficulty. A strong index of suspicion along with clinical and radiological correlation is essential to arrive at a correct diagnosis. In some bilateral cases, association with von Hippel-Lindau disease has been noted. Radical mastoidectomy and temporal bone resection, which may sometimes necessitate sacrifice of cranial nerves, is the treatment of choice.
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