A 35-year-old man with idiopathic eosinophilic fasciitis (EF) and morphea developed renal disease characterized by microscopic hematuria, nephrotic range proteinuria, and rapidly progressing hypertension, an association that has not previously been reported in EF. Initial clinical symptoms of EF began in July 1989; peripheral eosinophilia peaked at 30% in August 1990; an abnormal urinalysis was first observed in March 1992 and subsequently a renal biopsy was performed. Renal biopsy demonstrated focal segmental glomerulosclerosis and a subepithelial immune-type deposit. Partial fasciectomy and a course of methotrexate resulted in overall functional improvement of his extremities. Proteinuria and hematuria was reduced during methotrexate therapy.