Chondromyxoid fibroma (CMF) is a rare benign bone tumor, constituting less than 1%
of all primary bone tumors and typically affects long bones. It is an even rarer presentation
in the head and neck region and can prove diagnostically challenging. We report a
case of a 43-year-old woman diagnosed with a left-sided skull base CMF, an atypical
location for this tumor. The patient presented with progressive palatal pain without
any orbital symptoms. Imaging, including magnetic resonance imaging (MRI), revealed
a well-circumscribed lesion centered in the pterygopalatine fossa, extending into
the orbit toward the superior orbital fissure. An excisional biopsy demonstrated features
consistent with CMF. Surgical resection was subsequently performed via an endoscopic
transnasal approach, and follow-up imaging at six months showed no signs of recurrence.
In addition to the case presentation, we reviewed the existing literature on head
and neck CMF cases, focusing on clinical presentation, treatment options, and long-term
outcomes. While CMF is rare in this region, careful diagnostic and surgical management
is crucial due to the potential for misdiagnosis and recurrence. This case demonstrates
the benefits of minimally invasive endoscopic surgery, allowing for complete tumor
removal with minimal morbidity. Our report highlights the role of endoscopic approaches
in the treatment of skull base and orbital CMF and contributes to the growing body
of literature on the management of CMF in the head and neck region.