This report describes a patient with mixed connective tissue disease (MCTD) and trigeminal neuralgia (TN). Features of her TN, along with those of the 6 previously reported patients with TN and MCTD such as bilaterality and response to steroid therapy, suggest that the TN in MCTD differs from the idiopathic form of the disease. Because the TN often precedes other features of the MCTD, it may be useful to test patients with atypical TN for the presence of anti-RNP antibodies in order to identify a subset with both systemic disease and a potential to respond to corticosteroid therapy.