Pachypleuritis is a rare complication of pleural effusion due to nephrotic
syndrome. The causes of pachypleuritis vary widely and the impact on respir
atory function can be extraordinarily severe. A case is reported in an 11-y
ear-old girl with steroid resistant nephrotic syndrome and diffuse mesangia
l proliferation on the renal biopsy. She had chronic bilateral pleural exud
ates, which became increasingly abundant despite hemodialysis and ultrafilt
ration. Pachypleuritis with adhesions developed, causing a severe restricti
ve defect in ventilation. Bilateral pleural decortication was performed. Th
e clinical and functional outcomes were favorable. Pleural decortication is
effective in the exceedingly rare event of pachypleuritis complicating nep
hrotic syndrome.