Atypical pre-eclampsia cases are those that develop before 20 weeks of gestation and after 48 h after delivery and or have some of the signs and symptoms of preeclampsia without the usual hypertension or proteinuria which make them difficult to diagnose. Our aim is to report a case of atypical preeclampsia (before week 20 of gestation) associated with a HELLP syndrome and analyze the clinical features of atypical forms, assess differential diagnosis and highlight the progress in biochemical and biophysical markers that may help with diagnosis. Severe early pre-eclampsia (before 32 weeks of pregnancy) is associated with a risk of maternal mortality 20 times higher than after 37 weeks, and a higher risk of perinatal complications. Its symptoms are variable and reflect multisystem dysfunction. Despite the refinement of diagnostic tools available to clinicians, there are still clinical presentations that fall outside the definitions. Any good clinician must be aware of the atypical forms in order to initiate correct management without delay and thus avoid increasing maternal and perinatal morbidity and mortality.