Neuroimaging and neuropsychological studies have implicated the anterior temporal lobe (ATL) in sentence-level processing, with syntactic structure-building and/or combinatorial semantic processing suggested as possible roles. A potential challenge to the view that the ATL is involved in syntactic aspects of sentence processing comes from the clinical syndrome of semantic variant primary progressive aphasia (semantic PPA, also known as semantic dementia). In semantic PPA, bilateral neurodegeneration of the anterior temporal lobes is associated with profound lexical semantic deficits, yet syntax is strikingly spared. The goal of this study was to investigate the neural correlates of syntactic processing in semantic PPA, in order to determine which regions normally involved in syntactic processing are damaged in semantic PPA, and whether spared syntactic processing depends on preserved functionality of intact regions, preserved functionality of atrophic regions, or compensatory functional reorganization. We scanned 20 individuals with semantic PPA and 24 age-matched controls using structural and functional MRI. Participants performed a sentence comprehension task that emphasized syntactic processing and minimized lexical semantic demands. We found that in controls, left inferior frontal and left posterior temporal regions were modulated by syntactic processing, while anterior temporal regions were not significantly modulated. In the semantic PPA group, atrophy was most severe in the anterior temporal lobes, but extended to the posterior temporal regions involved in syntactic processing. Functional activity for syntactic processing was broadly similar in patients and controls; in particular, whole-brain analyses revealed no significant differences between patients and controls in the regions modulated by syntactic processing. The atrophic left anterior temporal lobe did show abnormal functionality in semantic PPA patients, however this took the unexpected form of a failure to deactivate. Taken together, our findings indicate that spared syntactic processing in semantic PPA depends on preserved functionality of structurally intact left frontal regions and moderately atrophic left posterior temporal regions, but no functional reorganization was apparent as a consequence of anterior temporal atrophy and dysfunction. These results suggest that the role of the anterior temporal lobe in sentence processing is less likely to relate to syntactic structure-building, and more likely to relate to higher level processes such as combinatorial semantic processing.