Association of cancer and thrombotic complications in acute leukemia is not uncommon. The underlying mechanism involved in thrombus formations is largely unknown with limited published data. Our patient was presented with complaints of continuous headache and persistent projectile vomiting. After initial tests, a magnetic resonance imaging of the brain performed revealed cerebral venous sinus thrombosis. Anticoagulant and anticonvulsant prophylaxis was initiated, and further treatment was planned. In such rare cases, early diagnosis and management are desirable. The role of clinicians in identifying the clinically suspicious signs and symptoms can help in identifying such rare conditions before developing severe thromboembolic complications. In the present study, we report a rare case of cerebral venous thrombosis, its associated treatment, and complications in a patient diagnosed with nucleophosmin gene-mutated acute myeloid leukemia. Similar Cases Published: None.