In this patient, we report a dramatic response to second line treatment with sunitinib, with complete resolution of two liver lesions at the time of first restaging.<h4>Conclusions</h4>This is the first report detailing a response to treatment with sunitinib of a gastrointestinal stromal tumor with an uncommon exon 12 PDGFRA mutation. Here, PDGFRA is linked to gastrointestinal stromal tumor.