This report describes a new case of the rare association of focal glomerulosclerosis with a peroneal muscular atrophy of the Charcot-Marie-Tooth type. The young patient was admitted with a nephrotic syndrome. A terminal renal failure rapidly developed. Numerous chronic nephropathies were known in the patient’s family. The ultrastructural study of the renal biopsy revealed a focal fusion of the epithelial foot processes, thickened and pleated mesangial basal laminae, vacuolated podocytes and small intranuclear clear inclusions. The Charcot-Marie-Tooth disease was of the hypertrophic type. According to a review of the literature and to further information concerning the follow-up of the 5 previously reported cases, the prognosis of the nephropathy is guarded with progression to end-stage renal disease in a few years. This case represents the 6th reported case of focal glomerulosclerosis associated with Charcot-Marie-Tooth disease.