Primary systemic amyloidosis is characterized by the deposition of insoluble monoclonal immunoglobulin light chains in various tissues and is usually associated with an underlying plasma cell dyscrasia. In the early stage of the disease, dermatological findings can be the only manifestation, as opposed to organ involvement in the later stages. A dermatologist can diagnose amyloidosis early with a skin biopsy stained with Congo red dye and other appropriate investigations. This case report describes a female patient with primary systemic amyloidosis confirmed histologically from a skin biopsy. When the diagnosis was established, cardiac involvement and monoclonal gammopathy were already present. Treatment with bortezomib and dexamethasone was initiated; due to side effects, the treatment was later switched to lenalidomide, which was better tolerated.