Cerebral venous and sinus thrombosis is a rare cerebrovascular disorder, which seldom represents a complication of acute promyelocytic leukemia (APL). As a part of the coagulopathy of APL, thrombosis is a less recognized and underestimated life-threatening manifestation and is overshadowed by the more obvious bleeding complications. Here, we described a 28-year-old woman with APL who developed massive thrombosis of the cerebral sinuses while on induction treatment with all-trans retinoic acid. On the basis of this report, the potential pathogenic mechanisms and the diagnosis based on magnetic resonance imaging (MRI) combined with magnetic resonance venogram (MRV) are discussed. Early anticoagulant therapy contributed to the progressive dissolution of the thrombosis, as documented by MRI, with the complete disappearance of neurological signs without sequelae. Given the increasing recognition of thromboembolic events in APL, the use of prophylactic anticoagulation during induction therapy may need to be redefined.