Pyoderma gangrenosum (PG) is a rare ulcerative skin condition. It can be associated with a number of systemic diseases. Association with monoclonal gammopathy of undetermined significance (MGUS) is uncommon, but prognosis may be different depending upon the type of MGUS. Cases of MGUS- related PG reported in the literature with data concerning evolution and treatment were identified through a PubMed search. A patient with recurrent PG in the setting of a MGUS-IgA-? in our department was also included. In total, 10 cases were identified. Only the two cases with Ig populations other than IgA improved without recurrence after treatment of the PG. All the patients with MGUS-IgA showed recurrences. Early multiple myeloma was proposed for three patients with MGUS-IgA-related PG. Second or third line treatments were necessary in some cases.