A 9-year-old girl with nephrotic syndrome visited a local hospital after developing fever, chills, and edematous changes and multiple hemorrhagic bullae on both legs over 2 days. Cultures of blood and an aspirate from the bullae yielded Vibrio vulnificus. The patient was transferred to our hospital because of persistent fever, generalized edema, acute renal failure, and disseminated intravascular coagulopathy. We treated this patient as a V. vulnificus infection complicated with necrotizing fasciitis. With minocycline and ceftazidime combination therapy was instituted. Emergency fasciotomy and continuous peritoneal dialysis were performed. The patient developed acute respiratory distress syndrome (ARDS) during the hospitalization, requiring intubation and mechanical ventilation. She eventually died. The histopathological findings showed diffuse alveolar damage with lobular pneumonitis. Hyaline membranes, composed of proteinaceous exudate and cellular debris, covered the alveolar surfaces. Microscopic examinations of lung could not distinguish the effects of cytolysin from other insults to lungs that occur in ARDS. This report highlights the postmortem pathological findings in V. vulnificus infection in a child with nephrotic syndrome complicated by ARDS.