Background: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor and is prevalent among children and adolescents. Surgery is the most important therapeutic approach for IMT and complete resection is recommended. Although 50% of IMTs present anaplastic lymphoma kinase (ALK) rearrangements, an efficacy has been shown by the use of Crizotinib . However the genetic landscape of this tumor is not fully understood and the therapeutic options are limited in particular for the remaining percentage of negative ALK tumors. Case presentation: In our case, we detail the clinical history of 18-year-old female patient diagnosed with pulmonary IMT negative for ALK, subjected to surgery and subsequently to follow-up. The initial pathology report oriented for a salivary gland lung cancer. Afterwards due to a second look by another pathologist an ALK negative IMT of the lung was diagnosed. We also perform a literature review based on IMT and other kinase fusions found in addition to ALK such as ROS proto-oncogene 1 (ROS1), rearranged during transfection (RET), neurotrophic receptor tyrosine kinases (NTRKs) and platelet derived growth factor receptor (PDGFR beta). Conclusions: IMT is a very rare disease involving children and adolescents. Little is known about the clinical and molecular characteristics, pathological diagnosis, prognosis and optimal management strategy of IMT. Since there is no "standard of care" therapy for IMT, identifying feasible genomic alterations could redefine the management of patients with negative ALK disease.