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Death is the definitive hallmark of amyotrophic lateral sclerosis and primary endpoint in most treatment study. Despite its importance limited data are available about the definitive causes of death in ALS nowadays. Previous autopsy studies pointed out that defining the cause of death based solely on a clinical examination is not a reliable method to reveal the true cause of death. It is unclear if treatments such as non-invasive ventilation (NIV) or percutaneous gastrostomy (PEG) have an impact on the cause of death. Seventy ALS patients had a complete macroscop-ic and microscopic post mortem analysis between 2003 and 2014. Viscera for the pathological causes of death and relevant concomitant diseases were also studied. Main causes of death were respiratory failure (69/70 patients). In 36/70, aspiration pneumonia and broncho-pneumonia led to death. 22//70 died of hypoxia without any signs of pneumonia and 5 patients requested assisted suicide inducing respiratory failure. Pulmonary embolism alone or in combination with pneumonia was detected in seven. Both bulbar (n=3) and spinal onset patients (N=3) had embolism without any clear correlation to mobility status. A single patient died from complication after PEG insertion. Average survival in patients using NIV was 7 month longer than without NIV. The proportion of aspiration pneumonia was significantly lower in patients with PEG (7/43 versus 7/26, p <0.0002). PEG had no effect on survival or BMI at death. Genetic testing could be performed in 32 patients prior to death. Disease-causing mutations (Superoxide Dismutase 1 (SOD1) or Chromosome 9 open reading frame 72 (C9orf72)) were found in 1/3 in this cohort. In this first prospective autopsy study, after establishment of the EFNS guidelines, NIV has a positive effect on survival. PEG insertion lowers the risk of aspiration pneumonia but has no effect on survival. Perhaps surprisingly, no correlation was observed between pulmonary embolism and ambulatory disability.