Objective This study aims to assess the therapeutic effectiveness of the AVDC/ICE regimen for children diagnosed with extracranial malignant rhabdoid tumors (MRT). Methods A retrospective analysis was conducted on 10 pediatric cases of extracranial MRT treated with the AVDC/ICE from January 2020 to January 2023. Results Clinical data from 10 pediatric patients with extracranial MRT were analyzed. The gender distribution was balanced (1:1), with a median age at diagnosis of 24.5 months (range 1-138 months). Three patients had distant metastases at diagnosis, predominantly in the lungs. The average size of primary tumors at diagnosis was (9.87±5.42) cm. Two patients with malignant rhabdoid tumor of the kidney (MRTK) experienced tumor rupture before surgery. Following pathological confirmation, all patients received the AVDC/ICE chemotherapy regimen. At the last follow-up, seven patients achieved complete remission (CR), one patient achieved partial remission (PR) and was receiving maintenance therapy with bevacizumab, sorafenib, and cyclophosphamide. Two patients with extra-renal extracranial rhabdoid tumor (EERT) succumbed to metastasis. The median follow-up was 14 months (range 5-28 months), with a one-year overall survival (OS) rate of 78.8% and a one-year event-free survival (EFS) rate of 68.6%. No severe treatment-related adverse events were observed. Conclusion The AVDC/ICE regimen emerges as a safe and effective chemotherapy option for managing extracranial MRT in pediatric patients.