AIM: This study described end-of-life care for children affected by spinal muscular atrophy type 1 (SMA1), which is characterised by progressive muscle weakness and develops in the first six months of life. METHODS: We retrospectively analysed 17 children (13 boys) who attended the University of Padua’s paediatric palliative care centre in Italy from March 2000 to March 2015. All the children received supportive care without proactive respiratory intervention to prolong survival. RESULTS: The median age at admission was 3.57 months, and the median age at death was 6.80 months. The most frequent symptoms were dyspnoea and pain. In the last 72 hours of life, 15/17 children required more intense doses of morphine and, or, benzodiazepines for intractable dyspnoea and pain, but deep palliative sedation was not needed. Airway suction to manage secretions and nasogastric tubes was required in all cases. The place of death was previously planned by the parents in all cases – home, hospital or hospice – and 15/17 deaths occurred in that place. We also interviewed 16 of the 17 parents after their child died. CONCLUSION: Our study found that symptom management and psychological support for families were the cornerstones of end-of-life care for children with SMA1.