Anaplastic Thyroid Carcinoma (ATC) is one of the most lethal tumours in humans, extremely rare in occurrence and very aggressive in nature. We hereby present a rare case of ATC with airway compromise. A 66-year-old male, presented complaining of a non-tender anterior neck mass rapidly increasing in size associated with dry cough, hoarseness and voice changes. Imaging studies revealed a large heterogeneous centrally necrotic lobulated left thyroid mass with metastatic lymph nodes and rightward tracheal deviation. Core biopsy and immunohistochemistry stains revealed a profile consistent with ATC. Patient's airway was compromised. Options for treatment and prognosis were discussed. Patient was discharged home with home hospice. A high index of suspicion for ATC is necessary in patients presenting with a rapidly enlarging neck mass. A prompt cytologic evaluation with metastatic work up is important to establish diagnosis. Due to its poor prognosis, an honest discussion regarding end-of-life issues must be initiated at diagnosis. Novel therapies toward genetic and epigenetic pathways have been developed, which is the basis of current clinical trials that are intended to improve clinical outcomes in the coming years.