Vasculitis syndrome accompanied by multiple liver lesions is uncommon. Biliary hamartomas, also called von Meyenburg complexes (VMCs), are small benign liver malformations that pathologically contain cystic dilated bile ducts surrounded by abundant fibrous stroma [1]. VMCs are considered to be congenital bile duct malformations. To date, there have been no reports of any cases of VMC complicated with systemic vasculitis syndrome. Here, we report an extremely rare case of VMC complicated with microscopic polyangiitis (MPA). A 59-year-old man was referred to our hospital with general fatigue, appetite loss, high fever, and numbness of the distal extremities in August 2010. He had been hospitalized in another hospital and had several examinations. Blood tests showed a high white blood cell count (WBC), elevated C-reactive protein (CRP) levels, and elevation of hepatobiliary enzymes. Abdominal CT showed multiple small hypodense lesions scattered in both lobes of the liver, which were suspected to be multiple liver metastases or microabscesses. Although two antibiotics (meropenem and clindamycin) were administered, all symptoms remained. After the detection of high myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) levels (185 EU) in his serum, he was transferred to our hospital for further treatment. His height was 168 cm and his body weight was 65 kg. On examination his body temperature was 37.7 C and his blood pressure was 134/89 mmHg. Physical examination revealed peripheral edema on both lower legs, and neurological examination revealed polyneuropathy on the distal extremities. A blood test showed elevated hepatobiliary enzymes and high levels of WBC and CRP. Laboratory values were as follows: WBC 33000/lL (normal 3800–9800/ lL); red blood cells (RBC) 375 9 10/lL (normal 440–540 9 10/lL); hemoglobin 11.2 g/dL (normal 14–17 g/dL); platelet count 42.6 9 10/lL (normal 15.5–36.5 9 10/lL); CRP 17.91 mg/dL (normal \0.2 mg/dL); asparate aminotransferase (AST) 119 IU/L (normal 10–35 IU/L); alanine aminotransferase (ALT) 73 IU/L (12–33 IU/L); alkaline phosphatase (ALP) 1215 IU/L (115–359 IU/L); c-glutamyl transpeptidase (c-GTP) 148 IU/L (normal 10–47 IU/L); total bilirubin 2.6 mg/dL (normal \1.1 mg/dL). Negative results were obtained for HBs antigen and HCV antibody. His blood urea nitrogen was slightly elevated (23 mg/dL, normal 9–21 mg/dL), and his serum creatinine level was within normal limits (0.57 mg/dL, normal 0.6–1.2 mg/dL). Urine test was positive for protein (?2) and blood (?3). The urine sedimentation test was positive for RBC casts (0–1/10), WBC casts (0–1/10), and granulocyte casts (0–1/10) per high-power field. Anti-nuclear antibody was negative (1:80, normal \1:160), but MPO-ANCA levels were elevated to 402 EU (normal \10 EU). Chest CT showed bilateral ground-glass opacities in both lower lungs. Abdominal enhanced CT showed multiple small hypodense lesions scattered in both lobes of the liver (Fig. 1). Abdominal ultrasonography (AUS) showed multiple small high-echoic lesions in the liver. MRCP demonstrated multiple small hyperintense nodules that were scattered throughout the liver but did not communicate with the biliary tree (Fig. 2). His clinical symptoms—lung shadows and high MPO-ANCA levels—led S. Sato (&) H. Watanabe T. Asano R. Saito H. Iwadate H. Kobayashi H. Ohira Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Fukushima 960-1295, Japan e-mail: shuzo@fmu.ac.jp