Purpose: The purpose of this study was to evaluate the association between dental anomalies of the permanent dentition and autism spectrum disorder (ASD). Methods: The dental and medical records and panoramic radiographs of 200 six- to 17-year-old subjects with a diagnosis of ASD were assessed retrospectively. The controls were 200 age- and gender-matched healthy children. The individual counts of shape, number, and positional dental anomalies and the total counts of all anomalies were recorded. Medical comorbidities were defined and documented as two subgroups: ASD subjects with (ASD-C) or without (ASD-NC) comorbidities. Results: Seventy-seven percent of ASD subjects had at least one comorbidity. Sixty-five percent of ASD subjects demonstrated at least one dental anomaly in comparison to controls (53 percent). There were no significant differences for the prevalence of number, shape, positional, or total anomalies between control, ASD-C, or ASD-NC groups. The ASD-NC group had a significantly higher proportion of pyramidal molars (P=0.02) and ectopically erupting teeth (P=0.04) when compared to controls. Conclusions: There were no significant differences in prevalence for shape, number, or positional anomalies in the permanent dentition between autism spectrum disorder subjects and healthy controls. The prevalence of pyramidal teeth and ectopic eruption was significantly associated with ASD.