Background: Sugammadex is a modified gamma-cyclodextrin that acts by selectively encapsulating free aminosteroidal neuromuscular relaxants. Several case reports have been published on the use of sugammadex in patients with neuromuscular disorders that include neuromuscular junction diseases, myopathies, neuropathies, and motor neurone disorders. The primary aim of this review is to systematically review the evidence on the use of sugammadex in patients with this heterogeneous group of diseases and provide recommendations for clinical practice. Methods: A systematic electronic search of Medline, Embase and CINAHL databases was done until June 2019, to identify case reports describing the use of sugammadex in adult surgical patients with neuromuscular disorders. Results: Of the 578 records identified through database searches, 43 articles were finally included for the systematic review. Of these, 17 reports were on patients with myopathy, 15 reports on myasthenia gravis, 9 reports on motor neuron diseases and 2 reports on neuropathies. Conclusions: Majority of the articles reviewed report successful use of sugammadex to reverse steroidal muscle relaxants, especially rocuronium, in patients with neuromuscular diseases. However, with sugammadex, unpredictability in response and uncertainty regarding optimum dose still remain issues. Quantitative neuromuscular monitoring to ensure complete reversal and adequate postoperative monitoring is strongly recommended in these patients, despite the use of sugammadex.