This work describes the diagnostic and therapeutic approach to a rare case of secretory carcinoma, initially diagnosed as AciCC, in a 30-year-old patient. This case of a minor salivary gland neoplasm was diagnostically controversial. Immunohistochemistry and identification of the fusion gene by molecular biology revealed the true identity of this entity.Due to the paucity of data available regarding treatment protocols and prognosis, further research is needed to establish a guideline for examination, medical and surgical management, and long-term outcome. MASC is a recently described salivary gland neoplasm that is defined by its close resemblance to secretory breast carcinoma, particularly at the genetic level where both tumors characteristically harbor the balanced translocation t(12;15) (p13;q25) resulting in an ETV6-NTRK3 fusion gene. Recognizing MASC and testing for the ETV6 rearrangement is not only a useful differential diagnostic tool but may be of potential value in treating patients.