OBJECTIVES: The Tracher Collins syndrome is a rare hereditary disorder, affecting approximately 1:40,000 to 1:70.000 people. It is characterized by abnormalities of the Pinna, hyperplasia of facial bones, antimongoloid slanting palpebral fissures with lower palpebral coloboma and cleft palate, and rarely associated with choanal atresia. This work aims at reporting a case of Treacher Collins as a child, associated with bilateral choanal atresia satisfactory surgery. CASE REPORT: Male patient, 10 years old, was admitted with complaints of chronic nasal congestion associated with mouth breathing. Mothers' reported mental delay, but interaction with social environment preserved. It was already submitted to surgery for correction of choanal atresia. To examination, we observed malformations of the Pinna, hyperplasia of facial bones, short palate, bilateral stenosis of external auditory canal, predominantly on the left. Nasal endoscopy showed stenosis of the right choanae. Immittance presented curve C in both ears, absent stapedial reflexes. Computed tomography and sinuses, showing proximal stenosis of external auditory canal, maxillary sinus and sphenoid pneumatized not, opacity range compatible with membrane partially occluding the choanae, reducing the amplitude of the right choanae. After last surgical correction of endoscopicaly choanal stenosis, the patient lies with improved airway patency and quality of life in multidisciplinary otolaryngologist follow-up, audiologist, pediatrician and neurologist. CONCLUSION: We sought to report a rare case of Treacher Collins associated with bilateral choanal atresia in children with good clinical response after correction of choanal atresia in a multidisciplinary approach contributing to the improvement of treatment.