Amyotrophic lateral sclerosis (ALS, also called Charcot disease, Lou Gehrig disease), is a progressive, neurodegenerative disease caused by the degeneration of motor neurons in the brain and spinal cord. There is no cure. This report describes a case of motor neuron disease with typical weakness in one leg, one hand and the tongue. Despite of treatment with riluzole, symptoms progressed relatively fast. Therefore, the patient decided to take cannabidiol (CBD, 2 × 200 mg/day) as co-medication, which was started 8 weeks after riluzole, and increased to a daily dose of 2 × 300 mg. Within 6 weeks, the impaired function of the right hand and foot reversed almost completely and dysphagia partially. Improvement was maintained for about 10 weeks, when again a slow progression of dysarthria and dysphagia was observed. Eighteen months after onset, speech is almost completely lost, and dysphagia also progressed. However, symptoms of the limbs (weakness, fasciculation, atrophy) worsened much less. It is concluded, that Co-medication with CBD may be able to slow down the progression of some but not all symptoms of motor neuron disease.