We observed that a metastatic GIST tumor harbored two different KIT mutations, including a deletion mutation in exon 11 (c.1675_1695del) and a missense mutation in exon 17 (c.G2446C, p. D816H), suggesting that acquired mutation in exon 17 occurred in the metastatic tumor compared with the primary tumor. Here, KIT is linked to metastatic neoplasm.