We report a patient with the glucagonoma syndrome and octreotide-resistant necrolytic migratory erythema (NME). The NME responded on two occasions to an intravenous infusion of essential fatty acids (EFA) and amino acids (AA). A deficit of serum EFA prior to treatment was corrected following the infusion, whilst plasma AA were low before and after treatment. These findings indicate that NME, in the glucagonoma syndrome, may respond to correction of the EFA deficit, and that NME may be a disease of EFA deficiency.