Background: Extrarenal malignant rhabdoid tumor (MERT) is a rare and highly metastatic tumor, which is more than 75% of patients dying within 6 months of initial diagnosis of MERT. Due to its rarity in clinical practice, there is no expert consensus or guidelines for the diagnosis, and it often leads to misdiagnosis and delayed treatment. Case: This paper reports a 16-year-old girl who presented with the chief complaint of acute abdominal pain. She underwent laparoscopic exploration and excisional biopsy, then pathological examination and immunohistochemistry revealed “extrarenal malignant rhabdomyoma”. One month after operation, she died of intra-abdominal hemorrhage and multiple organ dysfunction. Conclusion: Reviewing the cases of MERT admitted with acute abdomen reported in the last 10 years, it was found that these patients had a poor prognosis. The surgery and chemotherapy is usually beneficial to prolong the survival time of patients with MERT.