The FDA has approved pimicotinib (Pimjetmi) for adults with symptomatic tenosynovial giant cell tumors (TGCT) when surgery may cause worsening functional limitations or severe morbidity. The approval was based on results from Merck’s phase 3 MANEUVER trial. In MANEUVER, pimicotinib achieved an objective response rate at week 25 of 54% versus 3% with placebo, assessed by a blinded independent review committee using RECIST v1.1 criteria. The trial included 94 patients with confirmed symptomatic TGCT and measurable lesions. Safety updates include boxed concern areas around hepatotoxicity and embryo-fetal toxicity, alongside increased serum creatinine. The label recommends 50 mg orally once daily, positioning the drug as a once-daily medical option for a population with limited surgical benefit. The approval adds another targeted systemic therapy option for TGCT and may shift treatment discussions toward earlier medical management for patients deemed poor candidates for resection outcomes.