Aim and objective: To highlight the importance of looking for rare opportunistic as well as coinfections in children with primary immunodeficiency. Background: Opportunistic infections are often seen in children with primary immunodeficiency. Infections like nocardiosis and aspergillosis often mimic tuberculosis. So far, a single case of nocardiosis in hyperimmunoglobulin E syndrome (HIES) has been reported but none with coinfection with aspergillosis. Here we report a child with possible HIES presenting with nocardiosis and invasive pulmonary aspergillosis. Case description: A 3-year-old boy with recurrent abscesses and pneumonia presented to us with persistent fever and cough for 5 months, unresponsive to antitubercular therapy. Considering the suggestive history, characteristic facies, and elevated immunoglobulin E levels, HIES was diagnosed based on the NIH (National Institute of Health)-HIES score. High-resolution CT chest revealed mediastinal lymphadenopathy with right-sided nodular infiltrates and bilateral bronchiectasis. A positive serum galactomannan assay and growth of Aspergillus in bronchoalveolar lavage confirmed invasive aspergillosis. Mediastinal lymph node aspirate showed acid fast bacilli on smear and culture grew Nocardia species. Conclusion: It is important to look for rare opportunistic as well as coinfections in children with primary immunodeficiency. To the best of our knowledge, ours is the first report of pulmonary aspergillosis and nocardiosis in a child with HIES. Clinical significance: Albeit much rarer, aspergillosis and pulmonary nocardiosis can be close mimics of tuberculosis. Nonresolving pulmonary symptoms in the immunocompromised host must raise a suspicion of opportunistic infections including nocardiosis as early diagnosis and treatment may alter outcomes.