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HELLP syndrome is regarded as one of the most serious manifestations of preeclampsia, but approximately 15% of patients with HELLP syndrome have no clinical manifestations of preeclampsia; furthermore, although most cases of HELLP syndrome occur after 34 weeks, some cases occur at a much earlier gestational stage, even before 20 weeks of gestation, the underlying pathogenesis of “atypical” HELLP syndrome with very early onset or the basis of non-preeclampsia manifestations should be more actively explored. Obstetricians should carefully identify the potential etiology of “atypical” HELLP syndrome in order to provide a reasonable treatment and improve maternal and fetal prognosis.