Cohen syndrome is a rare genetic disorder consisting of truncal obesity, hy
potonia, mental retardation, microcephalia, characteristic facial appearanc
e and ocular anomalies. Other diagnostic clinical features include narrow h
ands and feet, low growth parameters, neutropenia and chorioretinal dystrop
hy, Acanthosis nigricans is a cutaneous disorder characterized by hyperpigm
entation and papillomatosis. Syndromal acanthosis nigricans may occasionall
y appear as a feature of several specific syndromes. We report a patient sh
owing the typical characteristics of Cohen syndrome with acanthosis nigrica
ns and hyperinsulinemia.