In our patient we found all these data, i.e., the presence of a monoclonal IgG lambda band in serum and urine, negative anti-PLA2R staining, a history of MGUS-IgGλ and the detection of a pathologic plasma IgGλ clone in BMA, associated with the histologic features already mentioned, led to the conclusion of a diagnosis of MGRS of an atypical NM type with IgG-λ deposition. The gene discussed is PLA2R1; the disease is nemaline myopathy.