After the antibiotic era, infectious causes of Gradenigo Syndrome (GS) have become rare, and other non-infectious etiologies, like granulomatous diseases and tumors, have been reported. Embryonic rhabdomyosarcoma (ER) is an aggressive malignant tumor that rarely affects the middle ear. Although infrequent, when it involves the petrous part of the mastoid bone it can present with facial pain in the distribution of the trigeminal nerve, ipsilateral abducens nerve palsy, and otorrhea. This triad of symptoms constitutes the Gradenigo Syndrome, which can be confused with complicated otitis media. For these reasons, neuroimaging is required for proper diagnosis. Treatment options are conservative (triple chemotherapy) and surgical. Leptomeningeal metastases is an unreported complication after surgical treatment of ER debuting as GS. Here, we present a case of ER debuting as GS which evolved to leptomeningeal compromise and death. The patient was treated with radical mastoidectomy in order to decompress the facial nerve. After the neurologic deficit improved but the clinical picture relapsed with a convulsive status that evolved to death. Our case highlights the need to weight treatment strategies considering conservative versus radical strategies, and to contemplate the possibility of complications after treatment.