This case report describes a diagnostic chain in a patient with atypical bilateral corneal opacity, which led to the diagnosis of a hematological disorder. The patient's medical history, clinical appearance and findings in confocal microscopy gave rise to the suspicion of a paraproteinemic keratopathy. The hematological laboratory diagnostics revealed a monoclonal gammopathy of the IgG kappa type. The bone marrow puncture led to the diagnosis of a lymphoplasmacytic lymphoma, which belongs to the group of B‑cell non-Hodgkin's lymphomas. This case demonstrates that paraproteinemic keratopathy can be associated with potentially life-threatening hematological diseases.
Keywords: Confocal microscopy; Immunotactoid keratopathy; Lymphoplasmacytic lymphoma; Monoclonal gammopathy; Paraproteinemic keratopathy.