To report endolymphatic sac tumor (ELST) as the initial finding in von Hippel-Lindau (VHL) syndrome.Case report.A 4-year-old boy presented in February 2001 with right hearing loss and seventh nerve palsy from ELST. Genetic analysis showed a VHL mutation (gene 3p25-26). Systemic and ocular examination for VHL syndrome-related tumors was unrevealing. Four years later, multiple retinal hemangioblastoma (retinal capillary hemangioma) were detected in both eyes and successfully treated with argon laser photocoagulation.ELSTs can be the initial manifestation of VHL syndrome. Lifelong screening for related tumors, particularly retinal hemangioblastoma, is advised.