The relationship between focal segmental glomerulosclerosis (FSGS) and pregnancy is complex and not completely elucidated. Pregnancy in patients with FSGS poses a high risk for complications, possibly due to hemodynamic factors, imbalance between angiogenic and antiangiogenic factors, and hormonal conditioning. Although poor clinical outcomes associated with collapsing FSGS are common outside of pregnancy, the prognosis during pregnancy is not well documented. We report 3 patients who developed collapsing FSGS during pregnancy, 2 of whom had presumed underlying FSGS. Two patients underwent biopsy during pregnancy, and 1, during the puerperium. None of the 3 patients improved spontaneously after delivery, and 1 experienced a rapid deterioration in kidney function and proteinuria after delivery. Aggressive immunosuppressive therapy led to a full response in 1 case (without chronic lesions) and to partial responses in the remaining 2 cases. These cases suggest that collapsing lesions should be considered in patients with FSGS who develop a rapid increase in serum creatinine level or proteinuria during pregnancy and that these lesions may at least partially respond to treatment.