Syringocystadenoma papilliferum is a rare hamartomatous adnexal tumor and appears mainly in the head and neck region. Rarely, such tumors may appear in the external auditory canal. There are only 14 described cases of the syringocystadenoma papilliferum in the external auditory canal in the Medline database. In this study, we present the first reported case of syringocystadenoma papilliferum arising in the modified radical mastoidectomy and occurring 12 years after ear surgery. A 26-year-old man was admitted to our clinic with complaints of discharge from the left ear, ear fullness, and hearing loss. These symptoms had been worsening for 3 years. Twelve years previously, he had undergone modified radical mastoidectomy by Bondy due to a cholesteatoma. Otoscopic examinations revealed otorrhea and a reddish mass measuring 1 × 0.8 cm arising from the tegmen tympanum and fundus of the mastoid cavity. A surgical biopsy was conducted with local anesthesia in the medical outpatient clinic, and it revealed syringocystadenoma papilliferum. A tumor resection was performed with a retroauricular approach. The bone attachment of the tumor was drilled out, and the ear cavity was covered with a mashed split skin graft. The ear cavity healed, and no tumor recurrence was observed during a 9-month follow-up period. Our case and literature analysis of previously described cases led to the conclusion that cases of syringocystadenoma papilliferum of the external auditory canal may be associated with different medical histories, age distributions, and origins than other syringocystadenoma papilliferums of the head and neck region. Tubular adenoma may occur together with syringocystadenoma papilliferum in 20% of cases. Malignant transformation of syringocystadenoma papilliferum may occur but is extremely rare. Computed tomography imaging may predict malignancy of the tumor. Tumors specific to the external auditory canal may occur in the modified radical mastoidectomy after middle ear surgery.