Scleromyxedema is a generalized and progressive fibromucinous disorder associated with substantial cutaneous and systemic morbidity.  The diagnosis is often challenging, as is management. We present here a patient with scleromyxedema with atypical, granuloma annulare-like histology, which contributed to delayed diagnosis and management, including a delayed workup for multiple myeloma.  Ultimately, the patient did well with appropriate therapy, but his presentation illustrates the importance of more widespread familiarity among dermatologists and dermatopathologists with this variant of scleromyxedema.