Sporadic but continuous single reports of simultaneous or synchronous gastrointestinal stromal tumors (GISTs) and epithelial carcinomas have been published in the literature, to the point where the question is raised as to whether there is a simple incidental association or whether these two types of neoplasms are connected by a pathogenic relationship [1]. GISTs are the most common mesenchymal neoplasms of the gastrointestinal tract, accounting for 0.1–3.0 % of all gastrointestinal malignancies. GISTs are considered the paradigm for targeted therapy in mesenchymal tumors and are also frequently associated (>95 % of cases) with mutations in either c-KIT proto-oncogene or platelet-derived growth factor receptor-alpha (PDGFRA) which determines the clinical response to imatinib mesylate [2]. The majority of GISTs occur as single lesions with no association established with tumors of other cell or origin except in patients with syndromes such as neurofibromatosis type 1 [1, 3]