Rationale: Ghost cell odontogenic carcinoma (GCOC) is a rare malignant odontogenic tumor with aggressive growth characteristics.
Patient concerns: A 41-year-old Chinese male visited our hospital in 2013, with a 4-month history of bloody purulent rhinorrhea with a peculiar smell in the right nasal cavity.
Diagnoses: The patient suffered from recurrent GCOC with suspected cholesterol granuloma of the maxillary sinus. The patient was incorrectly diagnosed with a calcifying epithelial odontogenic tumor at his first recurrence. Physical examination, radiological examination, and histopathology were used to identify GCOC.
Interventions: Considering the recurrence of GCOC and poor effects of single surgery, postoperative adjuvant chemotherapy and concurrent chemoradiotherapy were performed after radical surgical excision.
Outcomes: So far, no significant evidence has suggested recurrence or metastasis after a long-term follow-up.
Lessons: We advocate wide surgery with clean margins at the first accurate diagnosis. Combined modality therapy could be taken for the recurrent GCOC. We expect to provide a new treatment strategy to prevent the growth of this neoplasm.