Motor neurone disease (MND) is a chronic condition which presents mainly in the seventh and eighth decades. It classically presents with a mixture of upper and lower motor neurone features, with a predilection for the limb muscles as the presenting feature. The case report outlined below describes acute respiratory failure requiring non invasive ventilation (NIV), at the time of diagnosis of MND. It highlights the need for the acute physician to be vigilant in the differing forms of presentation of this condition and its subsequent diagnosis and management.