BACKGROUND Thestudy (descriptive cross sectional) was carried out between April. 2018 to Oct. 2018 in order to assess the knowledge of sickle cell anemia, attitude and perception towards premarital genotype counseling and screening among 190 youths of Dala LGA of Kano. METHOD A pre-tested, structured self-administered questionnaire was distributed to the respondents in their respective houses and schools. The sample were selected using simple random sampling. RESULT The study revealed that only 28.9% had good knowledge about Sickle cell disease (SCD). However, only 33% and 31% of the respondents knew their haemoglobin genotype and that of their serious fiancés respectively. Respondents gender appeared to have a significant influence on their knowledge (P<0.05) with males having relatively better knowledge than female. CONCLUSION There is a significantly deficient knowledge and perception about sickle cell disease among youths in Kano especially concerning the concept of the disease, mode of transmission and prevention. Increase in public awareness about the disease more especially among students in secondary and tertiary institutions as well as provision of free genetic screening, premarital counseling and testing, counseling and health education of the affected individuals need to be emphasized. In addition, government should regard the disease as an important public health burden and it should come up with national SCD control strategies that will aim at reducing the disease to the barest minimum.