Hematologic diseases are considered important contributors to cerebral venous sinus thrombosis (CVST) cases. This retrospective study aims to compare the difference of the clinical and radiological characters between CVST patients with and without hematologic diseases. Consecutive hospitalized CVST patients with hematologic disorders constituted the hematologic disorder group, while that without identifiable risk factors comprised the control group in this study. We systematically documented the various types of hematologic diseases associated with CVST, along with laboratory tests. Clinical manifestations, imaging findings, as well as treatment and prognosis, were recorded. A comparative analysis was conducted between the hematologic disorder group and the control group based on the aforementioned parameters. The final analysis included 97 CVST cases associated with hematologic diseases and 65 cases without any identified risk factors. The spectrum of hematologic diseases in our study ranged from iron-deficiency anemia to acute leukemia. Patients with hematologic disorder showed higher admission mRS, greater thrombotic burden, and higher incidence of stroke and cerebral hemorrhage (p < 0.05). The use of batroxobin significantly improved the prognosis of CVST caused by hematologic diseases, without causing major bleeding or death during the follow-up period. Patients with hematologic disorders who develop CVST tend to present with more severe conditions compared to those without identifiable risk factors. It is essential to conduct timely screening for CVST in patients with hematologic diseases who present with risk factors of thrombosis.