Neuromuscular diseases do not only affect a person’s physical, but also cognitive, emotional, behavioral and social functioning. Besides the muscle weakness, which is the main characteristic of the diseases, cognitive deficits, learning difficulties, neurodevelopmental disorders, emotional and behavioral problems and poor adaptive skills are often recognized in children and adolescents with neuromuscular diseases. Our goal was to investigate two psychosocial aspects of the diseases in Slovenian children and adolescents with neuromuscular diseases: adaptive skills, and emotional and behavioral problems. We compared 50 children and adolescents with different neuromuscular diseases, which we categorized into five groups of common neuromuscular diseases, with 50 healthy children and adolescents. For studying adaptive skills, we also included 248 children and adolescents from a normative study on adaptive skills. Adaptive skills were measured with the Adaptive Behavior Assessment System, Third Edition (ABAS-3), and emotional and behavioral problems were measured with the Child Behavior Checklist (CBCL). We found poor adaptive skills in children and adolescents with myotonic dystrophy, Duchenne muscular dystrophy and Becker muscular dystrophy in different areas of functioning. Those with spinal muscular atrophy showed poor adaptive skills only in domains where they need assistance due to their physical disability. In comparison with healthy peers, we did not find higher expressed emotional and behavioral problems in children and adolescents with neuromuscular diseases, except for higher social problems in groups with Duchenne muscular dystrophy and myotonic dystrophy. Yet the number of those in the borderline or clinical range suggests it is important to asses these problems, especially internalizing problems when working with this population. Our conclusions imply variety of comorbid psychosocial problems in children and adolescents with different neuromuscular diseases, based on specific diagnosis and many other contributing factors. To optimize psychosocial functioning and quality of life of children and adolescents with neuromuscular diseases, we recognize psychological support as very important.