The aim of this study is to profile the cognitive–linguistic performance of a male child (P.I.) with 22q11 deletion syndrome (22q11DS). Specifically, receptive and expressive language performance and nonverbal IQ (NVIQ) are described at two different time points—when P.I. was 6 and 10 years of age, respectively. Using case-based methodology, P.I.’s NVIQ and performance on global and structured language tasks are compared to typically developing children of the same chronological age and school-aged children with specific language impairment (SLI). The results show no improvement in NVIQ or vocabulary, but his morphosyntactic abilities did improve over time. The findings are discussed in relation to two hypotheses, either that the profile of language impairment in children with 22q11DS is distinctive to the syndrome or that there is co-morbidity with SLI. This is particularly important for speech–language therapists who have a primary role in diagnosing communication deficits and providing treatment.