A few rare syndromes have been delineated in which diabetes mellitus is inherited in association with other conditions. This is a case report of a Libyan boy who had diabetes insipidus, diabetes mellitus and optic atrophy, the coexistence of which though rare, is well recognised. During the past decade the literature reviewed was limited to cases having the 3 defects but more recently the diabetes insipidus, diabetes mellitus and optic atrophy have been described to be associated with deafness as well and is thought to be a-representation of a single genetic trait inherited as a mandelian recessive. The DIDMOAD Syndrome derives its name from the initials of the four major defects: DI-diabetes, insipidus, diabetes mellitus, 0A=optic atrophy, D=deafness.