Introduction: Lipofibromatous hamartoma (LFH) is a very rare, benign, slow-growing peripheral nerve tumor.
Case report: We present a case of LFH of the median nerve, which caused enlargement of the median nerve and the digital branches in a young male, and was treated surgically. Our treatment choice was resection of large portions of the fibrofatty tissue and decompression of the carpal tunnel.
Conclusion: A high index of suspicion for LFH is crucial in patients presenting with painless masses combined with acute compression neuropathy.
Keywords: Acute compression neuropathy; fibrolipomatous hamartoma; median nerve.