We report an autoimmune pancreatitis case with diverse manifestation of biliary stricture, lung fibrosis and immune thrombocytopenic purpura. The patient was a 70-year-old man who presented with jaundice. Abdominal ultrasonography and computed tomography revealed enlargement of the entire pancreas and endoscopic retrograde pancreatography showed diffuse irregular narrowing of the main pancreatic duct. Endoscopic retrograde cholangiography revealed a band-like stricture of the bile duct in the hilar hepatic region with intrahepatic duct dilation. Chest X-rays and computed tomography revealed interstitial fibrosis of the bilateral lower lungs. Thrombocytopenia and purpura appeared. Antinuclear antibody, anti-Ro (SS-A) and anti-La (SS-B) antibodies and rheumatoid factor were positive. All manifestations responded well to steroid therapy. From the present case it would appear that a multitude of manifestations are linked within one syndrome. If we encounter patients with autoimmune pancreatitis, we should not focus on a disease but rather on the possibility of a syndrome complex.