IMAGe syndrome is a rare condition, first reported by Vilain et al., in 1999, 
characterized by intrauterine growth restriction, metaphyseal dysplasia, 
congenital adrenal hypoplasia, and genital anomalies. Patients with this 
condition may present shortly after birth with severe adrenal insufficiency, 
which can be life-threatening if not recognized early and commenced on steroid 
replacement therapy. Other reported features in this condition include, 
hypercalciuria and/or hypercalcemia, craniosynostosis, cleft palate, and 
scoliosis. We report on a 7-year-old boy with IMAGe syndrome, who in addition to 
the features in the acronym also has bilateral sensorineural hearing loss which 
has not been reported in previously published cases of IMAGe syndrome. We 
discuss the clinical presentation in our patient and review the literature in 
this rare multisystem disorder.