A 26-year-old, HLA-B27 positive man with steroid dependent Crohn's disease presented with abdominal lymphadenopathy and multiple abscess-like masses in the spleen and liver 2 years after the initial diagnosis. A biopsy of one of the liver lesions showed epitheloid cell granuloma, no microorganisms were detected despite intensive investigations. The liver lesions and the lymphadenopathy repeatedly disappeared completely in response to prednisolone pulse therapy while the lesions in the spleen remained practically unchanged. One year later, on immunosuppressive therapy with azathioprine and low-dose prednisolone, the patient's condition exacerbated with development of fever, disseminated arthritides and enlargement of the splenic lesions. Following ineffective antimicrobial treatment, a splenectomy was carried out. Histology showed that the splenic parenchyma was filled with abacterial, rheumatoid granulomas. After a renewed flare-up of severe extraintestinal symptoms, the patient is now asymptomatic on immunosuppressive therapy with azathioprine and prednisolone. Abdominal lymphadenopathy is no longer present and the liver lesions are regressing. The intermittent course and the positive response to immunosuppressive therapy point to a further extraintestinal manifestation of Crohn's disease.