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We describe a case of ulnar nerve compression due to an anomalous muscle and a ganglion. The anomalous muscle arised from the tendon of palmaris longus and attached to both the pisiform bone and abductor digiti minimi muscle. Ulnar nerve was compressed at the proximal portion of the canal of Guyon. At operation, the anomalous muscle which seemed to be an accessory palmaris, and the ganglion were resected. The postoperative course was uneventful.

Distal ulnar nerve compression due to an anomalous muscle and a ganglion.