To describe the technique and outcome of a novel dual staged supraglottoplasty for the treatment of neurological induced adult onset laryngomalacia. A 55 year old male had a diagnosed neurodegenerative disorder with suspected Pompe's disease associated with Trap door epiglottis and proximal myopathy.This was complicated with emergency airway distress and subsequent tracheostomy.Trap-door epiglottis (also known as adult-onset laryngomalacia) associated with neurodegenerative disorders constitute a surgical challenge as it is often coupled with failure of tracheostomy decannulation when present. The patient underwent a novel dual staged endoscopic supraglottoplasty whereby an initial stage of epiglottopexy and submucosal diathermy was made at the vallecula.This was then followed by an interval of 6 weeks whereby a partial epiglottotectomy was made at the upper 3rd of the epiglottis and reduction of lingual tonsils was done using radiofrequency ablation.Trachesotomy was decannulated 1 month after the second stage procedure and his airway remains asymptomatic after 1 year of surgical treatment. This case report describes the success of tracheostomy decannulation after a novel dual staged supraglottoplasty for adult onset laryngomalcia (also known as trap-door epiglottis) .