Esthesioneuroblastoma remains a challenging disease because of its rarity, the complexity of surrounding structures, missing opinions of optimal treatment protocol, and complications associated with necessary surgery. Our objective was to analyse the management and outcome of a cohort of patients with esthesioneuroblastoma from 1990 to 2009 in a tertiary medical centre. There were 17 eligible patients (8 males and 9 females) with the median age of 53 years (range 20-75 years). An obvious inconsistency was noted in the management of the various tumours of the present series during the two decades due to a lack of a uniform treatment protocol. The median follow-up time was 57.5 months (range 3-158 months). Nine patients (seven with curative treatment intent) died of the disease with the median time from diagnosis to death of 60 months (range 3-161 months). Eight patients had no evidence of the disease at last follow-up visit (median 76 months, range 24-119 months). Recurrences were documented in seven of the patients. The median time from end of primary treatment to a recurrence was 57 months (range 6-110 months). The 5-year overall survival and disease-free survival was 68 and 62%, respectively. The management of ENB should be planned by an experienced head and neck surgeon as part of a multidisciplinary team in a tertiary referral setting. Multimodality therapy with long-term follow-up is preferable and should be set based on the available disease-specific classifications for clinical staging and histopathological grading.