Cystic fibrosis (CF) is considered one of the most commonly occurring fatal genetic disorders. This disorder is associated with pancreatic insufficiency and pulmonary complications. However, at birth the initial complications are associated with bowel obstruction. Cystic fibrosis management warrants an interdisciplinary team because this disorder affects various organ systems. Effective management of the newborn with CF assists in improving the child's overall prognosis. Family support is critical throughout the prenatal and postnatal periods. The case presented reviews a child born with suspected CF and the clinical course within the NICU.