: Pierre Robin sequence is defined by a triplet of clinical signs in newborns: micrognathia, glossoptosis and tongue-based airway obstruction often accompanied by U-shaped cleft palate. The reported incidence is ranging from 1 to 8.500 to 30.000 newborns. Therapeutic management of Pierre Robin sequence is based on the degree of the airway obstruction. A priori management of such cases can be extremely challenging due to the phenotypic plethora of Pierre Robin Sequence. A ten-day male newborn diagnosed with Pierre Robin was referred to our department for investigation and management of severe airway obstruction. Oxygen support was administered immediately and further examination revealed micrognathia and tongue profusion through the U-shaped cleft palate resulting total obstruction in the rhinopharynx and the nasopharynx resulting in severe dyspnea. Clinical examination and as well further investigation did not reveal further congenital abnormalities. Fiberoptic nasotracheal investigation that confirmed total obstruction of the upper part of respiratory tract was followed by tracheostomy due to signs of persistent respiratory insufficiency. Our report describes the successful algorithm for management of Pierre Robin syndrome as well as highlights the importance of fiberoptic intubation in such rare case.