We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.