The Crist-Siemens-Turens syndrome, or anhydrous ectodermal dysplasia is a rare condition. The authors present personal observation data of a 37-year old unmarried woman. From her childhood she suffered severe constipations, decreased hidropoiesis, hot flush. She had only two upper incisors. She developed partial alopecia of the head and eye-brow/eye-lashes and complete alopecia of armpits and pubic region, saddle-like nose, convex forehead and, so called, "Satyre" ears. Cranial X-ray revealed the presence of hypodontia. Gastroenteral X-ray, irrigoscopy and colonoscopy showed the presence of megadolicocolon. Treatment with purgatives gave no result. There were no literary data on the associated forms of the Crist-Siemens-Turens Syndrome and Megadolicocolon, previously.

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