An 8-year-old female golden lion tamarin (Leontopithecus rosalia) with a six-year history of hyperbilirubinemia presented with abnormal behavior including hunched posture, vocalizing while eating, lethargy, and decreased appetite. Serum chemistry confirmed severe, persistent hyperbilirubinemia, as well as markedly increased liver enzymes (ALP, ALT, and AST). Complete blood count revealed thrombocytopenia and a regenerative anemia, as well as a marked lymphocytosis. The lymphocytosis was most indicative of a chronic lymphocytic leukemia. A liver aspirate was non-diagnostic. On gross necropsy examination, there was diffuse bilirubin retention, micronodular cirrhosis of the liver, and diffuse osteoporosis. Histopathology of the liver revealed bridging portal fibrosis, biliary hyperplasia, severe cholestasis, and nodular regeneration. All bones examined had fibrous osteodystrophy, diffuse osteopenia, and osteomalacia. These findings are most consistent with hepatic osteodystrophy, which is characterized by chronic liver disease with secondary metabolic bone disease. This case report discusses the mechanisms of hepatic osteodystrophy, which has not yet been reported in veterinary medicine.