Bilateral endolymphatic sac papillary carcinoma

We report a case of bilateral and almost symmetrical endolymphatic sac papillary adenocarcinoma. A 22-year-old male patient presented with bilateral sixth, seventh, eighth and lower cranial nerve paresis and ataxia. Radiological investigations revealed extensively vascular tumours in the region of both jugular bulbs. The literature on this rare entity is briefly discussed.