Acute mastoiditis in a newborn complicated by the presence of facial nerve palsy is an alarming finding requiring rapid assessment and further investigation. Such an early presentation should point the clinician towards an underlying systemic pathology or congenital anatomical abnormality. Facial nerve involvement indicates severe infection and possible dehiscence of the facial canal. Although more frequent in children, it is rare in neonates. We would like to share our experience in managing the youngest known presentation of otomastoiditis at four days of life. The patient presented with otorrhea and facial paralysis and progressed to meningitis. He was finally diagnosed with chronic granulomatous disease.