Objectives of Investigation:Nemaline Myopathy (NM) is a rare syndrome with distinctive clinical and pathologic features and is characterized by abundant rod bodies within the muscle fibres. It presents generalized symmetric muscle wasting and weakness, typical facial 'fish mouth' appearance, frequent skeletal deformities and reduced or absent tendon reflexes. This case report describes a male child with NM-Syndrome who was 2 years 11 months at the time of the first examination in our department. He was referred from his speech therapist who had seen initial erosive lesions and caries on his anterior upper teeth. The aim of the case report was to present dental and orofacial status of this child with NM-Syndrome and exemplify dental and orofacial consequences in children with the disease. Materials and methods:2 year 11 month (in June, 2001) male child with NM-Syndrome. Clinical examination:Caries and plaque, extra- and intraoral photographs.Speech therapist examination. Starting prophylaxis by brushing his teeth and apply a fluorid varnish every 2-3 month. Results:We found erosive lesions on 52, 51, 61 and 62 (palatinal and incisal) which were caused by a longlasting reflux nearly one year ago. A poor masseteric and lip muscle function was recorded and was resulting in a permanent flow of saliva out of his mouth. After 7 month we observe a stagnation of the lesions. By undergoing a regular speech therapie, he now is able to control the periorale muscles for a short time und to close his lips. Conclusions:Children with NM-Syndrome should be considered as patients with high risk for caries and dental erosions. Consequently they need special preventive dental care. Additionally, there is also a need for oral motoric training for these patients Keywords: Nemaline Myopathy