Hepatic epithelioid cell granulomas that were negative for periodic acid-Schiff stain after diastase digestion were found in a 32-yr-old man who presented with painless hepatomegaly and slight fever. The patient never complained of intestinal symptoms, which in part explains why the diagnosis of Whipple's disease was made only 3 mo later, at a time when severe neurologic manifestations had appeared. The definitive diagnosis was made on the basis of the characteristic histologic findings in biopsy material obtained from jejunum and abdominal lymph nodes at laparotomy. The patient's condition, especially neurologic manifestations, rapidly improved after antibiotic therapy. It is noteworthy that Whipple's disease is generally not included among the causes of hepatic epithelioid cell granulomas. It is suggested that its possibility should be considered in patients with hepatic granulomas without obvious etiology, even in the absence of intestinal symptoms.