OBJECTIVE : Mechanical ventilation (MV) has been shown to improve survival and quality of life in motor neuron disease (MND). However, during the progression of MND, there may come a point when MV is no longer felt appropriate. Association of Palliative Medicine Guidelines have been recently published to help clinicians withdraw MV at the request of patients with MND in a safe and compassionate manner to ensure that symptoms of distress and dyspnoea are minimised. METHODS : In this report, we discuss the palliative and ventilatory management of six ventilator-dependent patients with MND who had requested the withdrawal of MV as part of their end-of-life care. RESULTS : We have withdrawn MV from six patients with MND at their request and our practice has been influenced by the Association of Palliative Medicine Guidelines. CONCLUSIONS : Withdrawal of MV in MND at a patient's request is challenging but is also a fundamental responsibility of healthcare teams. We discuss the lessons we have learnt which will influence our practice and help other teams in the future.