Ocular amyloidosis is infrequent, and the palpebral location is uncommon. It usually has a primary localized form, but can occur in systemic or familial amyloidosis. The diagnosis is mainly made by histopathology. Its treatment is surgical and the prognosis depends on clinical presentation and recurrences. We report the case of a 54-year-old woman with no medical history of the disease, who had bilateral isolated palpebral amyloidosis presenting as bilateral upper lid swelling with ptosis and corneal dystrophy. Diagnosis was confirmed by palpebral biopsy and the patient underwent excision of the involved lid tissue with reconstruction of the affected lid with cartilage autograft. Clinical progression showed improvement of the ptosis and the patient's comfort. Although rare, palpebral amyloidosis poses management problems, particularly in advanced cases, because of corneal complications and recurrence.