The aim of this paper was to enlighten the peculiar findings of the Pierre Robin sequence, a rare congenital anomaly with important afterbirth comorbidities. The management of this pathology requires highly specialised centers and highly qualified specialists in order to offer the best therapeutic strategy to the affected child. Early diagnosis and parental counseling play a key role in the managing of PRS: an earlier activation of the treatment path helps to yield better outcomes and to prevent complications. A case of a newborn affected by PRS is presented, showing the steps of the treatment strategy and the final outcome.