The diagnosis of peripheral nerve disorders in the neonate is challenging because of a wide range of conditions that can present with similar clinical features. The neonate with peripheral nerve disorders typically manifests generalized hypotonia with or without respiratory insufficiency. Diseases with lesions at various levels of the central or peripheral nervous system, as well as myriad systemic conditions, can all present with the same clinical features at birth. Neurophysiologic testing, appropriate genetic evaluation including detailed family history, cerebrospinal fluid analysis, and most importantly, careful neurologic examination and observation of the clinical course will help narrow down the differential diagnosis. This review aims to introduce representative forms of peripheral nerve disorders during the newborn period. We will discuss clinical features, how to differentiate between the disease entities, and the implications of early diagnosis and prognosis.