Carpal tunnel syndrome, in its idiopathic form, is an extremely common entrapment neuropathy in the clinical practice however secondary compressive causes are rare. Among secondary causes, tumors are even rarer. Although lipomas are the most common soft tissue tumor in the body, <5% of the benign tumors of the hand are lipomas. A 48-year old manual laborer man presented to us with a two-year history of numbness, tingling and burning pain in the palmar surface of the left hand and fingers along with a progressively increasing swelling in the hand and wrist. His medical history was unremarkable and no trauma episode was reported. According to the clinical examination and the result of median nerve conduction study (NCS) the diagnosis of carpal tunnel syndrome was established. Operative release of the transverse carpal ligament was subsequently performed along with excision of the lipoma using extensile open approach. Intraoperatively, median nerve and its digital branches were found to be stretched over the giant lipoma causing substantial compression to median nerve. Histopathological findings of the resected mass were consistent with lipoma. After two years the patient was pain-free without any sign of tumor recurrence. Lipomas are infrequently seen in hand and wrist, however giant lipoma as a cause of secondary carpal tunnel syndrome is even more rare, which makes this case interesting.