Abstract Background To investigate clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. Methods In our hospital, 305 patients with biopsy-proven IMN were divided into a non-focal segmental lesion group (FSGS- group) and focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. The general clinical and pathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. Results The FSGS+ group had longer disease course; higher blood pressure levels; and higher serum creatinine andβ2-microglobulin levels than the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, acute tubular lesion and chronic tubular lesion rates (all P< 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group(64.7% vs 82.2%) and was lower in association with calmodulin inhibitors than with cyclophosphamide in the FSGS+ group (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis(χ2=4.377,P=0.036). Risk factor analysis suggested that age at renal biopsy (P=0.006), 24-hour urinary protein quantity (P=0.01), chronic tubulointerstitial lesions (P=0.055), and FSGS lesions (P= 0.062) were risk factors for renal death; 24-hour urinary protein quantity was an independent risk factor for renal death. Conclusions Membranous nephropathy with FSGS is a risk factor for IMN but not an independent risk factor. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. Nonselective cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution. Key words Idiopathic membranous nephropathy; focal segmental sclerosis; cyclophosphamide; calmodulin inhibitor; prognosis;