Primary Malignant Peripheral Nerve Sheath Tumor of the Trachea: a Case Report with Brief Review of Literature Abstract Malignant schwannoma, also called malignant peripheral nerve sheath tumor (MPNST), is a rare and aggressive tumor arising from the nerve sheath. We describe a rare case of endotracheal malignant peripheral nerve sheath tumor occurring in a middle-aged male who presented with asthma-like symptoms for 6 months with progressively increasing dyspnea. A computed tomogram (CT) scan of the thorax revealed near complete luminal obstruction of the trachea by a mass lesion at the level of the second and third tracheal rings. Microlaryngotracheoscopy revealed a fleshy pedunculated growth arising from the left side of the second and third tracheal rings and obliterating almost the entire tracheal lumen. Intraluminal complete excision of the mass was done. Later, he underwent excision of the 2nd and 3rd rings after the histopathology revealed MPNST. Patient after 28 months of follow-up is free of disease.