Current practice guidelines state that enteral feeding should be proposed and provided whenever ALS patients show significant dysphagia and/or weight loss. Percutaneous endoscopic gastrostomy (PEG) is usually available at ALS centres, but this method is not indicated in the case of patients with severe respiratory impairment. We undertook a study to assess the feasibility of applying home parenteral nutrition (PN) to ALS patients with respiratory insufficiency, to assess the complications and survival times after the procedure. PN was provided, via an implanted port, to 30 ALS patients with respiratory insufficiency requiring nutritional support. Nutritional and neurological follow-up were carried out. The results were compared with those obtained on a group of 35 patients who underwent PEG, who were subdivided into patients with respiratory insufficiency and those without respiratory insufficiency at the time of the PEG. Post-procedure survival of PEG patients with respiratory insufficiency was similar to those of the PN group. The results of the study suggest that PN is a safe and effective method, which improves the patients' nutritional status and stabilizes their weight in most cases. Home PN could be applied to patients with advanced ALS and poor respiratory function as an alternative to enteral feeding.