Rhabdomyosarcoma (RMS) is a rare and aggressive neoplasm characterised by rapid growth and metastatic invasion. The most frequent localisation is the skeletal musculature of the limbs. The head and the neck are rarely involved. A 50-year-old woman presented to our attention because of a progressively increasing headache, ataxia and vomiting. MRI showed a lesion at the right cerebellopontine angle. Thereafter, the patient was submitted to a piece-meal removal of the neoplasm. Despite the postoperative MRI showed no signs of remnant, 7 months after the surgery, the disease recurred with multiple localisations, and the patient died a few days later. This report is the first description in the literature of a pleomorphic RMS of the cerebellopontine angle. This particular tumour carries a bad prognosis because of the vicinity of nervous structures and of the impossibility of achieving a one-piece resection. More than ever, the adjunctive treatments had to be effective against a potential remnant and in controlling recurrences.©2014 BMJ Publishing Group. All rights reserved.