Objectives Cerebral venous thrombosis (CVT) is a rare but potentially life-threatening condition in early pregnancy. We sought to describe the manifestation, treatment and outcomes of CVT presenting in early pregnancy through a retrospective consecutive case series in a tertiary referral center, so as to improve the awareness of this rare disease.Methods Between May 2018 and November 2021, consecutive seven patients with CVT presenting in early pregnancy were enrolled in Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University. All patients’ data like manifestations, laboratory and radiological data, treatment and prognosis were analyzed, and relevant literatures were also reviewed. Results All seven patients had been suffering from nausea and vomiting accompanied with headache prior to CVT occurrence. The headache was complained aggravation in severity with an average from 3.86 to 7.16 by visual analogue scale over several days. The mean age was 32.4±2.28 years, and mean BMI was 21.67 km/m2. All seven patients showed typical neurological symptoms on admission including epileptic seizures (n=2, 28.6%), limb weakness (n=4, 57.1%), aphasia (n=5, 71.4%), blurred vision (n=2, 28.6%), disturbed consciousness (n=3, 42.9%). Average score was 11.3 points (range from 3 to 15) on Glasgow coma scale (GCS). All patients were diagnosed with CVT at the first visiting day by imaging examinations and multiple venous sinuses thrombosis with average number of 3.7 per patient (range from 2 to 6) were confirmed, the most involved was transverse sinus (n=7, 100%), followed by sigmoid sinus (n=5, 71.4%). All of them was initiated anticoagulation treatment with low-molecular weight heparin (LMWH) instantly as soon as the CVT was defined. Two serious patients underwent catheter thrombolysis or/and decompressive craniotomy, but both still died of CVT deterioration within 5 days of admission. Other five patients were all performed selective induced abortion and was discharged after significant symptomatic improvement. They were remained anticoagulation for one year. At 12-month follow-up, MRI/MRV revealed sinuses were completely recanalized. Conclusions The present retrospective study should raise awareness of diagnosis and treatment of CVT in nausea and vomiting of early pregnancy complicated with headache. GCS evaluation on admission probably correlated with the prognosis. Neuroimaging remains the cornerstone for the diagnosis and instant anticoagulation prior to severe CVT aggravation is the key for the disease prognosis. Early pregnancy combined with CVT is not a contraindication of continued pregnancy based on the literature review.