The prevalence of IgE myeloma is 0.01% in all plasmocytomas. Although signs and symptoms are similar, it is generally accepted that IgE myeloma has a more malignant clinical course than other forms of plasmocytoma with monoclonal gammopathy. The disease has widely variable presentation. Most of the reported cases were accompanied by complications that are rarely seen with other types of myeloma. So far only one idiopathic (benign?) IgE monoclonal gammopathy has been reported. This case report is the first recorded IgE myeloma in Hungary with a rather unusual clinical presentation and diagnostic process. It is of particular interest that a monoclonal chain was detected in the urine of the patient several years prior to diagnosis.