Abstract The aim of this study was to describe the presentation pattern and challenges with the management of pediatric neuromuscular disorders (NMDs) in Ile-Ife, Nigeria through retrospective review of their clinical records. We recruited 24 children aged 14 days to 15 years with a male preponderance (male-to-female ratio = 2.4:1). Late presentation was a prominent feature which was highest among children with myopathies with a mean duration of symptoms of 46.5 ± 33.4 months before presentation. Peripheral neuropathy was the most common type of NMDs (63.0%). Among these, injection nerve palsy from the use of wrong site for intramuscular injections was the most common (60.0%). Nonavailability of electrophysiologic and genetic testing as well as appropriate medications was the major challenges. Acquired NMDs are common in our setting. There is a need to improve preventive measures of acquired NMDs and early diagnosis of the inherited types.