Forty-three patients with primary amenorrhea secondary to congenital absence of the vagina have been seen at this institution over the past 25 years. Associated urologic anomalies were present in 47% of patients in whom evaluation of the urinary tract was performed. Two patients recently presented with the rare combination of vaginal agenesis and solitary pelvic kidney, and one other such patient was found in a retrospective study of these records. A review of the literature concerning the associations of these conditions is presented with reference to frequency, embryology, pathophysiology, diagnostic methods, and various surgical procedures for correction of vaginal agenesis. The results of surgical correction using simple techniques in this institution have been comparable to those of other authors. The need for thorough preoperative evaluation of patients with genital malformations to include complete urologic studies is stressed.