Primary skin amyloidosis is a chronic skin disease in which amyloid deposits in the normal skin tissues without involving other organs. At present, the causes and mechanisms of morbidity have not been fully elucidated. There are few clinical reports of nodular skin amyloidosis, and the domestic reports are mostly limited cases. This study reported a rare case of a 46-year-old woman with primary localized cutaneous nodular amyloidosis (PLCNA). The patient presented with features of lymphatic malformation, a plexiform nodule of small blisters. Histological examination revealed amyloid deposits involving the superficial and deep dermis with a small number of plasma cells. Further examinations did not reveal evidence of systemic involvement, indicating a PLCNA. The presentation as lymphatic malformation lesions illustrates the importance of clinical pathology. Nodular amyloidosis typically manifests as single or multiple yellow-brown nodules or plaques of a few millimeters to several centimeters. The center of the nodule sometimes shows atrophy and relaxation or forms a bullous. It is recommended to perform a pathological examination to confirm the diagnosis to distinguish it from lymphatic malformation.