Microcytic hypochromic (MCHC) anemia with hemolytic components is common in clinical practice. Hemoglobinopathies and variants are one of the important underlying causes of MCHC anemia. The Indian population, by large, as various studies reported, showed a plethora of hemoglobinopathies with regional predilections for its types. The present systematic review is carried out for the evaluation of MCHC anemia for its underlying causes of hemoglobinopathies and their loco regional comparisons. The review was carried out by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method with selected keywords through the Google advanced search matchable to the objectives of the present systematic review. Upon the systematic review, it was observed that β thalassemia trait (βTT) remained the highest reported hemoglobinopathy. The other abnormal hemoglobin variants, though rare, also have been reported in the reviewed articles. It is concluded that patients with MCHC refractory to its regular treatment should be subjected to high-performance liquid chromatography (HPLC) in exclusion of underlying hemoglobinopathy and abnormal hemoglobin variants.