The metabolic derangement and resultant hypercoagulable diathesis associated with nephrotic syndrome (NS) has been clearly defined. Although venous thrombosis is frequently encountered in NS, arterial thrombosis is rare. This report describes an index case of a patient without known renal disease who presented with spontaneous arterial thrombosis of an upper extremity as the initial manifestation of NS. The case was complicated by subsequent deep venous thrombosis and pulmonary embolus in the absence of a patent foramen ovale. The patient was successfully treated with brachial artery thrombectomy followed by anticoagulation and immunosuppressive therapy. A renal biopsy was considered, but this would have required interruption of anticoagulation. This case illustrates the importance of performing a simple screening urinalysis for occult protein in patients with unexplained venous or arterial non-embolic thrombosis.