Primary tracheal neoplasia is uncommon in domestic animals and humans, and most reported tracheal tumors are malignant. To the authors’ knowledge, this case represents the third confirmed tracheal leiomyoma reported in dogs. Similarly, in human medicine, tracheal leiomyomas have only been reported sporadically and account for approximately 1% of tracheal tumors. A 12-year-old spayed female Pitbull was presented as an emergency because of worsening recurrent respiratory signs that were refractory to medical treatment. On initial evaluation, reduced mobility of both arytenoid cartilages was observed, and left arytenoid lateralization was therefore performed. However, the patient continued to experience severe episodes of respiratory distress requiring orotracheal intubation. Subsequent bronchoscopy revealed an intraluminal tracheal mass partially obstructing the airway lumen. Because of this obstruction, an emergency temporary tracheostomy was performed caudal to the mass. Once the patient was clinically stabilized, computed tomography was performed to complete surgical planning. Surgical treatment consisted of segmental tracheal resection and end-to-end anastomosis, with removal of 24% of the total tracheal length. Histopathological examination confirmed the diagnosis of tracheal leiomyoma. Complete surgical excision was achieved, supporting a favorable prognosis.