Learning Objectives: This paper was to determine the characteristics of facial nerve primary tumors misdiagnosed as tumor-free conditions many years prior, and to identify appropriate treatments.The cases of five Chinese patients with misdiagnosed primary tumors of the facial nerve were reviewed; in each case, the condition had been misdiagnosed more than 8 years prior. All patients presented with progressive or complete facial paralysis and hearing loss, with or without vertigo. We reviewed pre- and post-operative images (including CT scans of the temporal bone) and MRI data. After review, all tumors were completely resected using the translabyrinthine or transmastoid approach and were confirmed to be primary tumors of the facial nerve.All tumors were totally resected. Facial-hypoglossal nerve anastomosis failed in one patient whom we sought to manage in two stages, because fibrosis developed at the end of the facial nerve. One patient accepted two-stage facial-hypoglossal nerve anastomosis and patient status improved to House-Brackmann (H-B) grade V from H-B grade VI. The other three patients chose not to undergo reconstruction. All patients recovered well, with no other complications evident after follow-up periods of 0.5–3 years.Unusual primary tumors of the facial nerve should be considered in patients with progressive facial paralysis, especially if this is accompanied by hearing loss or vertigo. Misdiagnosis creates operative difficulties, diminishes the chance of facial nerve reconstruction, and increases the likelihood of poor reconstructive outcomes.