Absence of the uterus, right ovary, and right fallopian tube were demonstrated by pelvic pneumography and confirmed on laparoscopic examination of a patient with primary amenorrhea and the Turner phenotype. A small rudimentary ovary and fallopian tube were present on the left. Chromosomal constitution was 46,XX. This is a rare example of the association of absence of the uterus with the Turner phenotype in a female with apparently normal chromosomal constitution. Other cases with similar findings may help resolve the question as to whether the association was a chance occurrence or due to a genetic anomaly responsible for both the Turner phenotype and regression of the Müllerian structures.