A case report of Chronic Myeloid Leukemia (CML) with extreme thrombocytosis. A premenopausal women with an age of 45-year-old was admitted with chief complaints of fatigue and generalized body weakness, upper abdominal pain and two episodes of recent gum bleeds without fever or other bleeding manifestations. No other comorbidities. On Examination of US Abdomen gross splenomegaly and mild hepatomegaly are observed. On investigation, patient was also observed with extreme thrombocytosis (10,500,000/mm3 ) and leuk ocytosis with moderate anemia. By the above investigation of leukocytosis, patient was investigated for CML and found to be positive for BCR-ABL by Reverse Transcription PCR (RT-PCR). And prescribed with imatinib 400 mg/day and achieved a complete hematological response by the end of 3 months. On evaluation, patient responded well to imatinib treatment. In above case of CML, atypical presentation of disease was identified and treated with kinase inhibitors.