Infections and/or immune-mediated reactions may occur after intravesical instillation of bacillus Calmette-Guérin for the treatment of bladder carcinoma. We report herein a cryoglobulinemia vasculitis occurring after intravesical BCG instillation for a superficial papillary transitional cell bladder carcinoma. The patient, an 80-year-old man, presented peripheral ischemia 10 days after the second course of intravesical BCG instillation. Biological evaluation revealed autoimmune thrombocytopenia, hypergammaglobulinemia, low C3 and C4 complement fraction levels related to mixed cryoglobulinemia and lupus anticoagulant. The patient was treated with heparin and prostacyclins with a good outcome. All of the immune anomalies spontaneously regressed within 3 months. To our knowledge, cryoglobulinemia has only been reported once in the literature and lupus anti-coagulant has never been reported as a complication of intravesical BCG instillation.