Background and Aim: Coronary artery disease is a multifactorial disease caused by various risk factors including genetic factors. MTHFR A1298C polymorphism is one of the important genetic factor have clinical contribution. Due to lack of information on association of MTHFR A1298C polymorphism with CAD, we aimed to assess the risk of MTHFR A1298C polymorphism with CAD in patients from Haryana Materials and Methods: The study includes 97 CAD patients (56 males, 41 females) and 93 healthy controls (males 51, females 42) in between 21–50 years of age. The risk factors, lipid profile and clinical parameter were recorded. DNA was isolated from blood and MTHFR A1298C polymorphism was screened by PCR–RFLP. Results: No significant difference (p>0.05) in the distribution of MTHFR A1298C polymorphism has been observed in CAD patients in comparison to healthy controls. No significant difference (p>0.05) in the distribution of MTHFR A1298C polymorphism among young age (≤40 years) CAD patients demonstrate no independent association of MTHFR A1298C polymorphism with the early onset of the disease. No significant difference (P>0.05) in the incidence of AA, AC and CC was observed in CAD patients with positive family history. Conclusion: The MTHFR A1298C polymorphism does not associate with the development of CAD in patients from Haryana and therefore, rule out possibility of independent risk factor confer CAD. The incidence of MTHFR A1298C polymorphism does not differ among CAD patients with age, sex and positive family history