Hyperimmunoglobulin E syndrome (HIE) is a rare condition characterised by marked elevation of serum IgE level, chronic dermatitis, intense pruritus, and recurrent serious infection. The major organism is usually S aureus. We report a case of an infant with HIE, who had pulmonary nocardiosis. The clinical features, immunological abnormalities, and radiological features of the condition are described. The child finally succumbed to the complications of pulmonary nocardiosis.