We report about an infant who was diagnosed with spinal muscular atrophy type 1 (Werdnig-Hoffmann) at the age of 7 weeks. In a detailed discussion with the physicians the parents decided against mechanical ventilation in the event of either acute or chronical respiratory failure. During care at home all palliative actions were taken to optimize the quality of life for the child. The boy died at the age of 7 months at home. Whether continuous mechanical ventilation in a child with spinal muscular atrophy type 1 should be the individual decision of the parents or whether it should never be offered by the physicians is discussed controversially in the present medical literature.