OBJECTIVE: To present a case of bilateral choanal atresia in newborn, and to review existing literature on the subject. CASE REPORT: Newborn, male, 6 hours of life, born at term, attended by a pediatrician with respiratory distress and was diagnosed clinically bilateral choanal atresia. Transferred to the otolaryngology department of the São José of Avai Hospital carrying Guedel cannula. Performed Computerized Tomography of the facial sinuses, confirming the clinical diagnosis of bilateral choanal atresia and showing the mixed nature of atresia. Directed endoscopic surgical treatment for correction of bilateral choanal atresia, a catheter in both nostrils. RN in the was maintained in the ICU under mechanical ventilation and intubated on the 11th postoperative day. RN progressed well and was discharged after 62 days in hospital in good general condition with satisfactory weight gain, endoscopy showing satisfactory patency of neocoane. Underwent echocardiography, electrocardiography, laboratory tests, and ultrasonography of the urinary tract, without evidence of any amendment. CONCLUSION: Choanal atresia is a congenital failure of development of communication with the nasal cavity and nasopharynx occurs in approximately 1:5000-8000 births, affecting twice as many of male sex. It may be unilateral or bilateral being potentially fatal. Congenital anomalies may be associated with up to 50% of cases, CHARGE (C, coloboma, H, congenital heart disease; A, choanal atresia, R, retarded growth and development, G, genital anomalies, E, ear abnormalities and deafness). Bilateral atresia should be treated as early as possible.