The long pentraxin 3 (PTX3) is an important element of the innate immune system and has potential as a diagnostic tool in inflammatory conditions. We studied PTX3 in patients admitted to an intensive care unit with severe meningococcal disease and compared it with the short pentraxin C-reactive protein (CRP). Twenty-six patients with meningococcal disease were studied, 17 patients presented with meningococcal septic shock (shock group), and 9 patients presented with meningococcal meningitis or bacteremia (no-shock group). Pentraxin 3 and CRP were measured by enzyme-linked immunosorbent assay. High plasma concentrations of PTX3 (median, 579 μg/L) were seen at admission in patients with meningococcal disease. Concentrations were significantly higher in patients with shock compared with patients without shock (medians, 801 and 256 μg/L, respectively; P = 0.006). In contrast, CRP at admission was lower in the shock group as compared with the no-shock group (medians, 58 and 165 mg/L, respectively; P = 0.008). High PTX3 and low CRP concentration at admission discriminated between presence and absence of shock (area under the receiver operating characteristic curve, 0.85; P = 0.007 for PTX3 and area under the receiver operating characteristic curve, 0.84; P = 0.01 for CRP). PTX3 did not correlate with disease severity (pediatric risk of mortality) and days spent in the intensive care unit. PTX3 at admission and PTX3 peak concentration both showed a negative correlation with plasma fibrinogen concentrations. C-reactive protein concentration at admission correlated negatively with disease severity. In conclusion, PTX3 was an early indicator of shock in patients with severe meningococcal disease that followed a pattern of induction distinct from CRP.