Nephrotic syndrome and monoclonal gammopathy of undetermined significance are thought to be associated with venous thromboembolism. However, the association is thought to be weak and is often ignored by clinicians. We present a rare case of sudden-onset bilateral pulmonary emboli with lower extremity deep vein thrombosis in a patient diagnosed with both minimal change disease and immunoglobulin M (IgM) kappa monoclonal gammopathy of undetermined significance. No previous report has been published describing venous thromboembolism in a patient with plasma cell dyscrasia and minimal change disease. This case establishes the importance of considering a diagnostic workup for both disorders in patients with venous thromboembolism. Furthermore, venous thromboembolism risk in patients with both of these diseases is significant. Benefits of prophylactic anticoagulation in these patients are still controversial.