The streak gonad syndrome is described in detail with a reveiw of the literature and a report on the diagnosis of 38 additional patients. Certain signs and symptoms of the syndrome are always present, ie, female phenotype, primary amenorrhea, infantile breast status, sparse public and axillary hair, infantile external genitalia and vagina, atrophic vaginal smear, immature uterus, high serum follicle-stimulating hormone (FSH) level, low urinary estrogen, retarded ossification, osteoporosis, and the streak gonad. Therefore, they are considered to be cardinal features. Certain somatic anomalies known as associated stigmata occur with varying frequency in accordance with the karyotype. Thus, patients with the streak gonad syndrome can be classified into groups according to their chromosomal complements. It is believed that the term streak gonad syndrome instead of Turner's syndrome, gonadal dysgenesis, or other terms is a much more accurate description of the syndrome, at least from the clinician's point of view.