Background: Compression of the ulnar nerve at the level of Guyon’s canal is a very rare compressive neuropathy. Due to the vast range of symptoms that can manifest depending on the degree of ulnar nerve compression, the clinical picture is not consistent. Objective: The aim of the study is to outline the diagnostic techniques and therapeutic options. Case report: We reported a case of ganglion cyst-induced compression of the ulnar nerve in Guyon’s canal. A 45-year-old female patient underwent surgical ulnar nerve release in Guyon’s canal at the Clinic for Plastic and Reconstructive Surgery. Discussion: After a thorough medical history and physical examination, the diagnosis of the syndrome is made, and ultrasound and magnetic resonance imaging (MRI) testing are used to determine the origin of the neuropathy. A ganglion cyst was identified pathohistological one month following the surgical excision of the soft tissue tumor. In order to hasten the patient’s nerve recovery, physical therapy was recommended, and the patient was monitored for the following two years. After two years of treatment, the patient has made a very good recovery of the functionally damaged hand, as determined by a modified Bishop scoring method for evaluating functional ulnar nerve recovery. Conclusion: In virtually all cases, early surgical intervention can lead to an outstanding functional recovery. If the symptoms are more severe and continue or get worse for more than three months, early surgical intervention is the gold standard for treating Guyon’s canal syndrome. If soft tissue formations are compressing the ulnar nerve in Guyon’s canal, MRI is thought to be the gold standard for diagnosis.