Background: Hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is a lifethreatening pregnancy complication. Various mechanism may lead to diffuse endothelial damage which causes multi-organ dysfunctions, with possible brain involvement. We report a rare case of postpartum HELLP syndrome associated with posterior reversible encephalopathy syndrome (PRES) and conducted a literature review from the PubMed, Embase, and Cochrane databases to summarize the clinical, laboratory and radiological characteristics. We found only 10 papers describing this association occurring during pregnancy and 7 articles in the post-partum.Case presentation: Our patient developed brief hypertensive peaks followed by two episodes of generalized tonic-clonic convulsions during postpartum. Brain magnetic resonance imaging allowed the diagnosis of PRES and an association with HELLP syndrome was established after blood exams. After medical therapy, the follow-up was marked by full recovery. Conclusion:Patients with PRES do not always show typical manifestations and MRI is crucial for its diagnosis. Cases of postpartum PRES with involvement of anterior brain regions after HELLP syndrome are rare but must be considered and they should be kept in mind as a differential diagnosis in women presenting with seizures, headache and altered mentation. Clinicians should be aware of this neurological syndrome because prompt diagnosis and treatment may result in complete resolution.