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Authors present a case of ulnar nerve compression due to a rare combination of anatomic variations, aberrant branching pattern, and FCU insertion at the wrist, which posed a diagnostic and therapeutic dilemma. The literature related to isolated distal ulnar motor neuropathy and anatomic variations of the ulnar nerve and adjacent structures is also reviewed. This case demonstrates how anatomic variations can complicate the interpretation of clinical and electrodiagnostic findings and underscores the importance of thorough exploration of the nerve in consideration for possible variations. Clin. 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