A boy who had been diagnosed with chronic granulomatous disease (CGD) at the age of 6.5 years had a medical history of multiple bacterial infections, including pneumonia, staphylococcal liver abscesses and septicemia, from birth. At the age of 10 years and 4 months he developed an infection that was accompanied by high fever and pulmonary, mediastinal and paravertebral infiltrations. Aspergillus niger was cultured on bronchial secretions obtained by bronchoscopy. Shortly thereafter, proteinuria manifested and progressed to the nephrotic level. A skin biopsy indicated a diagnosis of amyloidosis. An anti-fungal treatment with amphotericin B and other agents, along with surgical pus drainage, intravenous leukocyte mass, interferon-gamma and immunoglobulin infusions, was ineffective, and the patient eventually died from multi-organ failure. The postmortem examination revealed the presence of disseminated aspergillosis and systemic amyloidosis. Although no direct evidence is available that would confirm the causative role of aspergillosis in the development of systemic amyloidosis, to the best of our knowledge this is the first report of a CGD case with complications of both invasive aspergillosis and systemic amyloidosis.