Alström syndrome is a complex pleiotropic disorder caused by mutations in ALMS1. Systemic characteristics of this disorder include truncal obesity, hyperinsulinemia, and T2DM, acanthosis nigricans, short adult stature, and vision and hearing loss, dilated or restrictive cardiomyopathy (infancy or adolescence/adulthood) with CHF, hypothyroidism and hypogonadism. The gene discussed is ALMS1; the disease is hyperinsulinism.