A 63-year-old male patient presented with retrobulbar pressure which had been present for 1 year. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) revealed symmetrical retrobulbar, perivascular and retroperitoneal infiltration of soft tissue and also showed cardiac involvement. In combination with the histological findings displaying infiltration by foamy histiocytes, Erdheim-Chester disease was diagnosed.