INTRODUCTION: The hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome usually occurs in association with preeclampsia (PE). Risk factors and short-term and long-term outcomes of women with PE with HELLP (PE-HELLP) or isolated PE (iPE) are unclear. METHODS: Using data from the Cohort of Cardiovascular Diseases in Pregnancy (CONCEPTION) (a French nationwide, population-based, prospective cohort) study, all women who gave birth at ≥22 weeks of gestation between 2010 and 2018 were included. The main outcomes were mortality, cardiovascular, cerebrovascular, thromboembolic events, and renal failure (RF). RESULTS: Among the 2,829,700 women with first deliveries, 73,736 women with iPE, 9147 women with PE-HELLP and 2,746,817 women controls were studied. Diabetes, chronic hypertension, and obesity were more frequently associated with iPE than PE-HELLP. Social deprivation was positively associated with iPE but negatively associated with PE-HELLP. Chronic inflammatory diseases and lupus were more associated with PE-HELLP than iPE. During the first year, iPE and PE-HELLP constituted major risk factors for hypertension and all other major adverse events versus controls; however, PE-HELLP was a significantly stronger risk of major adverse cardiovascular events (MACEs), stroke, thromboembolism, and RF than iPE. After the first year, iPE remained significantly associated with all outcomes, whereas PE-HELLP was a risk factor only for hypertension and RF versus controls; the risk of RF was similar for iPE and PE-HELLP. CONCLUSION: iPE and PE with HELLP differ regarding their risk factors and types and dynamics of their major complications.