Hemicrania continua (HC) is a primary headache disorder, characterized by unilateral, unremitting headache of moderate to severe intensity, associated with ipsilateral autonomic signs and an absolute response to indomethacin. We report a case of a 34-year-old woman who presented with strictly left-sided unremitting headache whose initial symptoms suggested a temporomandibular disorder, but the patient was ultimately diagnosed with hemicrania continua. The patient displayed an absolute response to a trial of indomethacin but she had to quit soon due to gastric intolerance and vertigo. Then topiramate was prescribed which she responded completely. This case and the other reports as well suggests that the differential diagnosis of temporomandibular disorders and HC is challenging. HC generally needs to be treated lifelong and indomethacin may not be a good choice because of its side effect profiles while topiramate may be considered as an alternative. The IHS criteria of HC need to be revised to provide a broad based description thus providing new therapeutic options.