We report a rare case of cervical dermal sinus associated with a dermoid cyst in a 10-month-old infant, who presented with a 1-month history of motor weakness of the right upper extremity. Magnetic resonance imaging showed an intradural extramedullary dermoid cyst extending from C3 to C4, which was connected with the skin dimple along the sinus tract. Total excision of the dermoid cyst and the sinus tract with C3-5 laminectomy resulted in good functional recovery.