Purpose: NUT carcinoma is a genetically defined, highly aggressive and frequently fatal squamous cell carcinoma. It is characterised by a translocation of the NUT gene on chromosome 15q14 and often presents as a rapidly growing tumour of the midline upper airway or skin. Methodology: A retrospective case report of a 45 year old male presenting with a rapidly growing postauricular lump. The lump had increased in size four-fold within 2 months and an initial biopsy revealed a poorly differentiated squamous cell carcinoma. Preoperative staging PET/CT identified locoregional nodes to the ipsilateral neck but no distant metastases. Results: The patient underwent a resection, radical neck dissection and reconstruction with an anterolateral thigh flap. Initial histological examination of his specimen identified a predominantly undifferentiated carcinoma with limited sarcomatoid and squamous differentiation. An immunohistochemical study did not identify further atypical findings and the specimen was subsequently sent to a quaternary cancer centre for a second opinion where a NUT1 fusion mutation was identified by RNA fusion assessment. Three weeks postoperatively patient deteriorated and a repeat staging scan revealed new metastases to his liver and bilateral lungs. Conclusion: Recent gene sequencing studies suggest that NUT carcinoma is less rare than previously thought and there emerging targeted therapies that have made this pathology potentially survivable without surgical resection. The available NUT specific antibody immunohistochemical stain is highly sensitive and specific and should be performed liberally in cases with poorly differentiated and rapidly growing carcinoma.