Inflammatory myofibroblastic tumor (IMT) is a rare, mesenchymal low-grade neoplasm characterized by benign histology but has the potential of showing aggressive clinical behavior with local invasiveness and recurrence. It primarily occurs in children and young adults in the lung and abdominopelvic regions, but there are reports of oral cavity IMTs. In this report, the authors present a case of a neonate born with an enlarging tongue mass whose biopsy revealed IMT with anaplastic lymphoma kinase (ALK) positivity. Treatment included tumor debulking and an ALK inhibitor, crizotinib, which resulted in complete remission. To our knowledge, this is the only reported case of oral cavity IMT with ALK positivity in a neonate.