RESUMEN
The present study reports a rare case of capillary hemangioma of endolymphatic sac. A 23-year-old male who underwent von Hippel-Lindau disease presented with recurrent sudden sensorineural hearing loss. Magnetic resonance imaging revealed a heterogenous enhanced mass in the right endolymphatic sac, which was hyperintense on the enhanced T1-weighted images and inhomogenous on the T2-weighted images. Pre-operatively, this tumor was believed to be an endolymphatic sac tumor because of the history of von Hippel-Lindau disease. During the surgery, vascular tumor was removed by transmastoid approach. A histopathological examination indicated that the tumor was a capillary hemangioma. To the best of our knowledge, the present study is the second case of hemangioma in the endolymphatic sac and first case of von Hippel-Lindau disease.