: Leyden-Möbius muscular dystrophy is an autosomal recessive hereditary disease of unknown aetiology; it is a congenital disorder of protein metabolism primarily affecting proximal muscle groups leading to progressive muscular dystrophy. It later spreads to the muscles of the pelvic floor and lower extremities. The estimated incidence is 1:200,000. This paper describe a case of pregnancy and delivery in woman with progressive Leyden-Moebius muscular dystrophy. Cesarean section was performed due to progression of the underlying disease. First postoperative day DIC occure and surgical revision of abdominal cavity was performed. Although the uterine suture was strong, diffuse bleeding was present. Blood was not coagulating. Supravaginal amputation of the uterus was performed including left-sided adnexectomy due to bleeding from the left ovarium. Due to the severity of the condition and assumed necessity of long-term controlled ventilation, the patient was transferred to the intensive medicine department. She was dismissed home after 91 days of hospitalisation. Gravidity in advanced muscular dystrophy is rare and associated with a high risk. Due to muscle weakness, diaphragm weakness, atrophy of individual muscle groups, spine deformities and often dislocation of thoracic organs, these patients cannot avoid the caesarean section to end their pregnancy, followed by prolonged intubation and controlled ventilation. During pregnancy, the growing uterus elevates the diaphragm and impairs breathing. Therefore, pregnancies in such patients will probably always have to be ended prematurely.