Pseudo-Kaposi's sarcoma which develops due to arteriovenous fistulae for haemodialysis is relatively well known. In contrast, nail changes associated with pseudo-Kaposi's sarcoma or venous hypertension are rare. We report a novel case of pincer nail deformity associated with pseudo-Kaposi's sarcoma and venous hypertension, complications of an arteriovenous fistula for haemodialysis, and review eight similar cases reported in the literature. Most of the subjects presented with similar findings, having circulatory disturbance due to an arteriovenous fistula and/or increased venous pressure, and swelling, discoloration and papules/nodules of the skin distal to the shunt. Of the nine patients, three had overcurvature of the nails, in which the lateral edge of the nail pressed deeply into the lateral nail fold. Pincer nail deformity associated with pseudo-Kaposi's sarcoma after placement of an arteriovenous fistula may be relatively common and should be recognized as a specific sign of circulatory disturbance due to the arteriovenous fistula.