Objective. To report a case of a patient with Nemaline Myopathy (NM), focusing on the main complications and the proceeding of physiotherapy. Method. This study focused on the clinical evaluation and major complications, assessment, treatment and the results of physiotherapy. The patient´s motor evaluation was conducted according to the Motor Function Measure (MFM) scale and the version of the International Classification of Functioning, Disability and Health for children and youth (ICF-CY). The maximal inspiratory pressure (PIMÁX) was assessed using a digital manovacuometer. Physiotherapeutic interventions were made daily, during 180 days. Results. The results of the MFM scores were: D1-17, 9%; D2-66, 6%; and D3-76, 1%; in total 48.9%. In ICF-CY is observedlimitationsinthepatient's postural transfers,nodifficulty maintainingbody positionexceptstanding, moderate walking difficulty and severe difficulty in moving an object with the lower limbs. At the end of the hospitalization period PIMÁX test was conducted, revealing a 36cmH20 inspiratory muscle strength. Conclusion. This report provides an understanding of the clinical evolution and prognosis of NM compared with the literature. The physical therapy was essential to enable the return of functional and recreational activities and to assist the child in respiratory dysfunctions.