OBJECTIVECraniosynostosis is a congenital skull deformity that impacts development and quality of life of children if left untreated. This study aimed to evaluate literature regarding presentation, treatment, and outcomes of craniosynostosis in Africa.METHODSA systematic review of the literature using PubMed/MEDLINE, Scopus, Web of Science, and Google Scholar databases was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.RESULTSFourteen retrospective/prospective studies with 620 patients and 14 case reports involving 27 cases (8 countries) were included. In 12 articles, 56.6% of patients (317/560) were males, with a mean age of 2.4 years (confidence interval [CI]: 1.1-3.7). Abnormal head shape was the most reported presentation in 77.8% of cases (332/427, 8 articles). Syndromic craniosynostosis was seen in 25.2% (CI: 13.7%-36.6%). Common phenotypes were trigonocephaly in 31.5% (CI: 3.6%-59.4%), anterior plagiocephaly in 23.2% (CI: 5.1%-41.3%), and scaphocephaly in 22.1% (CI: 13.5%-30.8%). Five hundred seventy eight patients, 99.5% (CI: 99.0%-100.0%), underwent surgical treatment. Vault remodeling was performed in 72.9% patients (CI: 47.4%-98.6%). Postoperative complications included cerebrospinal fluid leaks 5.4% (CI: 0.0%-11.6%) and surgical site infections 4.5% (CI: 0.0%-10.8%). Follow-up ranged between 0.2 and 40.9 months; 95.6% of cases (CI: 90.1%-100.0%) exhibited improved deformity and neurological deficits at last follow-up. The mortality rate was 3.1% (CI: 0.0%-6.9%, 2 articles).CONCLUSIONSFew studies on craniosynostosis in Africa highlight the need for more research. Treatment with open techniques yields few complications and a low mortality rate. Early diagnosis and collaborative data reporting will enhance understanding of its burden and variations across Africa.