Purpose: Spinal cord metastasis from rhabdomyosarcoma (RMS) is extremely rare, with three cases reported to date. Herein, we report an aggressive case of RMS of the infratemporal fossa who which developed spinal cord metastases during treatment. Case presentation: A 6-year-old girl presented with an enlarging painless mass around her right ear for 3 months. An enhanced magnetic resonance imaging (MRI) revealed a 5 × x4 × x4.5 5 cm mass on her right infratemporal fossa. A tru-cut biopsy was performed, and histopathologic examination revealed the diagnosis of rhabdomyosarcoma. At the time of the diagnosis, cerebrospinal fluid cytology was negative for malignant cells. The patient underwent induction chemotherapy. There was minimal response to chemotherapy, and the patient underwent curative radiotherapy. However, by 12th fraction of RT, the patient developed a progressive weakness on her lower extremity. Spinal MRI revealed multiple gross masses in different parts of the spinal cord. The local radiotherapy was changed toas craniospinal radiotherapy. However, two 2 weeks after the completion of the RT, the patient developed sepsis and expired because of septic shock. Conclusion: Parameningeal RMS is a peculiar subgroup of RMS, which needs an aggressive approach. Despite aggressive approach, meningeal spread is the most important cause of the treatment failure. We should keept in mind that during the treatment, there can be meningeal spread towards to either the brain or spinal cord; therefore, we should follow -up the patients closely from this aspect.