Epidermoid cyst occurring within the tongue is rare. The aim of this article is to discuss the management of an adult patient who presented with respiratory obstruction due to this congenital epidermoid cyst within the tongue. A 32-year-old male patient who was brought to the accident and emergency department of the University of Port Harcourt Teaching Hospital, Port-Harcourt, Rivers State, Nigeria was presented. He came with a history of difficulty in breathing as a result of a slow growing swelling of the tongue which started from birth. There was associated pain, difficulty in speech and mastication as the swelling increased in size. Detailed physical examination, sonographic imaging and histopathology were carried out. On examination, patient was acutely ill-looking, in severe respiratory distress, febrile, mildly pale and dehydrated but anicteric. Extra-oral examination revealed mildly protruded upper and lower dento-alveolar arch as well splaying and recession of the anterior and posterior teeth of the upper arch. Intra-oral examination revealed moderately tender, fluctuant and grossly enlarged tongue obstructing the oro- and nasopharyx. A diagnosis of dermoid cyst was made and the patient was booked for surgery after resuscitation. Excision of the cyst was done under general anaesthesia/naso-tracheal intubation by an intra-oral approach. Ultrasound was able to give a thorough assessment of the cystic nature of the swelling while post-operative histopathology was used to confirm the specific type of teratoma. The relationship of the cyst to the intrinsic and extrinsic muscles of the tongue and mylohyoid muscle was relevant to the surgical approach to the lesion.