Three cases of chromatin negative gonadal dysgenesis were reported. The first case was one of pure gonadal dysgenesis with a chromosomal constitution of 46 (XY). The second case had in addition a short stature, diabetes mellitus and extreme deuteranomaly. The third case had short stature, neck webbing, had menstruated regularly for 6 years, and had a chromosomal constitution of 45 (XO).