Verbal IQ deficits are frequently reported for school-age children with sickle cell disease (SCD), yet the profile of language abilities in SCD is unclear. We examined semantic, syntactic, and phonological processing in five-to-seven-year-olds at high neurologic risk based on SCD subtype (N = 33), at low neurologic risk with SCD (N = 21), and without SCD (N = 54). Low-risk SCD did not show language processing deficits. High-risk SCD showed deficits in all three language domains. Language processing deficits in SCD at the start of middle childhood are related to neurologic risks and include language skills beyond vocabulary.