BACKGROUND: Trigeminal neuralgia (TN) caused by a tentorial dural arteriovenous fistula (TDAVF) is quite rare. To date, only 10 cases and 2 small case series have been reported in this regard, and most were treated with either embolization or surgery. Here, we report a unique case of a TDAVF presented as TN, which was embolized with Onyx first and resected later. CASE DESCRIPTION: A 57-year-old male presented with right-sided TN. Magnetic resonance imaging revealed a variceal venous dilation occupying the right lateral pontine cistern and multiple venous flow void signals adjacent to the right trigeminal nerve root entry zone. Digital subtraction angiography revealed the right TDAVF, which was completely embolized with transarterial Onyx later. The patient remained symptom free for 1 year before TN recurred. Digital subtraction angiography did not exhibit the recurrence of fistula. After resection of embolized dilated veins, the symptom alleviated and the patient remained symptom free for the 5-month follow-up to date. CONCLUSIONS: Even complete obliteration of fistula could cause the recurrence of neuralgia, and resection of embolized dilated veins might be effective for the treatment of TN in such recurrent cases.