Abstract
Amyloidosis consists of a spectrum of disease ranging from primary localized forms to secondary forms due to systemic disease. Amyloidoma, a variant of localized amyloidosis, is the least frequent manifestation of tissue amyloid deposition, which can be either amyloid A (AA) or amyloid light-chain (AL) type. Soft tissue amyloidoma is uncommon and mostly affects the abdomen and mediastinum and rarely affects the extremities. We present a middle-aged woman who developed a tumor-like swelling in the extremity, in which comprehensive investigations revealed features of primary amyloidosis with no evidence of internal involvement, representing a rare presentation.