This case report describes a patient with a metastasised olfactorial esthesioneuroblastoma Hyams grade 4 who has been treated with debulking surgery and radiotherapy. After relapse in lymph node, lung and brain, he received additional irradiation and six cycles of carboplatin, vincristine and cyclophosphamide intravenously every three weeks. The patient has now been disease free for 7.8 years. Our data suggest that metastatic esthesioneuroblastoma is sensitive to platinum-based chemotherapy. This patient illustrates that this tumour is very sensitive to platinum-based chemotherapy and that durable complete response can be achieved, even in a metastatic ENB.