Objective: Examine differences in clinical presentation between males and females referred to a tertiary care hospital for autism spectrum disorder (ASD) evaluation. Method: 66 children (52 males, 14 females) between the ages of 4 and 14 (M = 98.92 months, SD = 26.72 months) referred for possible ASD completed a comprehensive evaluation including a standardized measure of intellectual ability, as well as the Autism Diagnostic Observation Schedule (ADOS) for a subset (n = 26). Results: There was no difference in the age at which males and females were evaluated (t(64) = .67, p > .05). Of the 66 children, 26 (21 males, 5 females) received a diagnosis of ASD (39.4%). There was no difference in age (t(24) = 1.57, p > .05) or cognitive abilities (t(24) = 1.79, p > .36) between males and females. Common comorbid diagnoses were intellectual disability (n = 10), ADHD (n = 5), and language disorders (n = 6). For those children not given an ASD diagnosis, the most common alternative diagnoses were ADHD (n = 18), intellectual disability (n = 8), and language disorders (n = 4). These did not differ between genders. Conclusion: We did not find evidence to suggest the age at which males and females were evaluated for ASD differed in our participants. Additionally, differences in cognitive abilities were not evident. Given the clinic setting, it is possible the nature of our referrals differs from those in community settings. Additional findings regarding the nature of social difficulties between males and females will be discussed.