We aimed to identify the contemporary prevalence of byssinosis through a systematic review. We used Medline, Web of Science, Embase, and Global Health databases to identify studies published in any language between 2000 and 2019, reporting primary data on byssinosis among adults. We used the Joanna Briggs Institute checklist to estimate the risk of bias in studies and undertook a qualitative, narrative data analysis. The review considered the prevalence of byssinosis, chest tightness, and airflow obstruction in textile workers in low- and middle-income countries (LMICs). We found 26 relevant studies that included 6930 workers across 12 countries. Most of the studies (n = 19) were from Asia, and 7 from African countries. Twenty-five studies were cross-sectional surveys while 1 was a cohort study. The prevalence of byssinosis was reported by 18 studies, and ranged from 8% to 38%, without any clear associations, at the group level, between the prevalence of byssinosis and durations of workers’ exposures. Prevalence of chest tightness ranged between 4% and 58% and that of airflow obstruction between 10% and 30%. We found a strong correlation (r = 0.72) between prevalence of byssinosis and cotton dust levels. Our findings indicate that byssinosis remains a significant, contemporary problem in some parts of the textile sector in LMICs.