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Introduction: Inflammatory myofibroblastic tumor (IMT) is a rare entity, classified within soft tissue sarcomas. It is an intermediate malignancy tumor, which seldom presents as metastatic disease. The treatment of choice is surgery, except in cases where surgery is not possible due to localization or if it presents with metastatic disease. Approximately 50% of IMTs will exhibit ALK translocation, providing a therapeutic target for these patients., Case Presentation: A case is presented of a patient with metastatic IMT in complete response to treatment with alectinib, maintained for over 4 years., Conclusion: This case showed a long time complete response in patient with IMT treated with alectinib., Competing Interests: Johana Benedetti Pedroza has collected research funding for clinical studies (institutional) from PharmaMar, Eli Lilly and Company, AROG, Bayer, Eisai, Lixte, Karyopharm, Deci-phera, GlaxoSmithKline, Novartis, Blueprint, Nektar, Forma, Amgen, and Daichii-Sankyo. Irene Carrasco García has received personal fees for advisory board, consulting, and travel expenses from Pharmamar. She has also gotten research funding for clinical studies (institutional) from PharmaMar, Eli Lilly and Company, AROG, Bayer, Eisai, Lixte, Karyopharm, Deci-phera, GlaxoSmithKline, Novartis, Blueprint, Nektar, Forma, Amgen, and Daichii-Sankyo. Gala Martínez Bernal has obtained research funding for clinical studies (institutional) from PharmaMar, Eli Lilly and Company, AROG, Bayer, Eisai, Lixte, Karyopharm, Deci-phera, GlaxoSmithKline, Novartis, Blueprint, Nektar, Forma, Amgen, and Daichii-Sankyo. Lastly, Isabel Miras Rodriguez has obtained research funding for clinical studies (institutional) from PharmaMar, Eli Lilly and Company, AROG, Bayer, Eisai, Lixte, Karyopharm, Deci-phera, GlaxoSmithKline, Novartis, Blueprint, Nektar, Forma, Amgen, and Daichii-Sankyo., (© 2024 The Author(s). Published by S. Karger AG, Basel.)