Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytic disorder. The diagnosis was based on the relationship between radiologic findings, clinical manifestations, and pathologic features of the bone biopsy. We report a case of ECD with unusual presenting symptoms: a 56 year-old man presented with cough, abdominal pain, and recurrent episodes of headache associated without any seizures. Peculiar computer tomography (CT) findings were key for the diagnostic suspicion. Bone biopsy and other radiological investigations confirmed the diagnosis. CT findings can help raise the suspicion of ECD. CT is easy to perform and widely available in comparison with kinetic cardiac magnetic resonance imaging and nuclear imaging. Therefore, CT findings of ECD can reduce the therapeutic delay between diagnosis and therapy prescription.