The aim of this study is to demonstrate the spectrum of radiologic findings of hyperimmunoglobulin E (hyper- IgE) syndrome that is relatively rare in adults. We describe the imaging findings of this rare disease in five patients. The study group consisted of 4 men and 1 woman. The final diagnosis was made according to laboratory findings in all cases. We detected lung involvement in 3 cases, paranasal sinus involvement in 4 cases and vascular involvement in 1 case. Soft tissues were involved in 5 cases. Bone involvement was demonstrated in 3 patients. The most frequent radiologic finding was widespread infectious involvement of soft tissues, bones and neighboring structures such as bursae and tendons and respiratory system.