Background Carpal Tunnel Syndrome (CTS) is the most prevalent peripheral nerve entrapment disease. Its pathophysiology is multifactorial and defined as idiopathic in most cases. We present a rare case of CTS secondary to tumoral calcinosis and then searched the English literature to present the details of all published cases with this entity. Case presentation. A 52-year-old woman presented for a one-year history of numbness and paresthesia in her right hand. The patient’s signs, symptoms, physical examination, and nerve electrodiagnostic testing suggested median nerve compression at the level of the carpal tunnel. However, a confirmatory magnetic resonance imaging of the wrist showed a localized calcareous lesion in the carpal tunnel. Subsequently, carpal tunnel release and mass excision were successfully performed with no recurrence at a 3-month interval. Conclusion CTS secondary to tumoral calcinosis is a rare benign condition. Physicians should remain vigilant and include it in their differential diagnosis when facing a previously healthy patient presenting for chronic CTS symptoms.