Objectives 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Results We have withdrawn MV from six patients with MND at their request and our practice has been influenced by the Association of Palliative Medicine Guidelines.

Conclusion 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.