An observational study conducted on patients with Systemic Lupus Erythematosus (SLE) at a tertiary referral centre of Northern Kerala in South India had shown that hematological manifestations as the most common initial presentation of the disease. It was observed that 82% of the patients had hematological manifestations either alone or with another problem at presentation. If we consider the organ or tissues initially affected as the seat of the disease, SLE is a Hematological disorder because it can present more often with hematological manifestations alone. Even in those who present with features of musculoskeletal, skin or other system involvement, many have coexisting hematological problems. In cases with hematological abnormalities as the predominant or only manifestation of the disease, the diagnosis may be delayed or missed at the time of presentation, if the index of suspicion is low or if there is improper and inadequate follow up. One of the common coexisting abnormalities in patients with initial hematological presentation was autoimmune hypothyroidism, which is not included in the American College of Rheumatology (ACR) criteria for diagnosis of SLE. The most prevalent hematological abnormality was anemia which was due to multiple mechanisms. There was an inverse association of arthritis with hematological manifestations. A significant number of patients did not satisfy the ACR criteria at the time of diagnosis but did so on follow up. ACR criteria are weak to diagnose such patients and therefore needs revision. We also propose an alternative to ACR criteria as “The Kozhikode Criteria for SLE”.