Background: We present the case of a 24-year-old woman with respiratory distress associated with a cyst of the fourth branchial cleft that displaced and compressed the upper airway, so the cervical mass was surgically resected, the patient recovered completely. Case presentation: We present the case of a 24 year old female no other pertinent medical history who presents to the emergency department of way outpatient to respiratory distress associated with a cervical mass. A computed tomography (CT) scan shows a right cervical cystic mass that was displacing and compressing the upper airway. A total resection of the cystic mass was performed, after which the patient recovered completely. The histopathological analysis indicated a branchial cleft cyst which, due to its location, was thought to be the fourth branchial cleft, a rare congenital anomaly. Conclusions: Fourth branchial cleft cysts are rare malformations. They should be taken into consideration in the differential diagnosis of cervical masses in young adults, especially in situations of potentially life threatening airway compromise where an emergent procedure should be performed to guarantee the patient's life, the diagnosis is based on an adequate history and physical examination, with the support of imaging studies, with CT scan imaging being preferred as it provides information for surgical planning. Treatment is based on complete resection of the cystic mass, which relieves the symptoms of mass effect and decreases the risk of recurrence.