Cancer related stroke may have different phenotypes from non-cancer stroke, especially in terms of stroke progression and recurrence. We performed a case-control study to identify their incidences and risk factors in cancer related stroke. Between January 2001 and December 2009, we conducted a retrospective review of acute ischemic stroke patients with cancer who were admitted to Seoul National University Hospital, Seoul, Korea. The stroke patients without cancer served as control. We collected demographic variables, vascular risk factors, stroke phenotype, clinical course, and cancer information including diagnosis, stage, and treatment status. Among cancer stroke patients, the potential risk factor of stroke recurrence was evaluated. The mean age of the 102 cancer patients was 66.4 ± 10.8 years, and 64.7 % were men. The mean time interval from cancer diagnosis to stroke onset was 39.7 ± 60.9 months. The principal lesion pattern of cancer stroke was multiple dots extending single vascular territory (39.2 %), and they were associated with low hemoglobin and high fibrinogen levels. Stroke progression and recurrence were noted in 9.8 and 27.5 % of cancer stroke patients, and in 9.3 and 12.7 % of control patients, respectively. The stroke subtype was independently associated with recurrence of cancer stroke after multiple logistic regression (odds ratio = 3.165, 95 % confidence interval = 1.080-9.277, p = 0.036). Cancer related stroke has a distinct phenotype in terms of infarction pattern and laboratory findings. Stroke recurrence is frequently observed among cancer stroke patients, and its risk is related with stroke subtype.