Seckel syndrome presenting with complete heart block

Seckel syndrome is a rare autosomal recessive disorder characterized by facial dysmorphic features known as bird-headed dwarfism. Only about 100 cases have been reported. Cardiac anomalies have been described as a potential association with Seckel syndrome. We report a 21-year-old woman with Seckel syndrome and epilepsy who presented with status epilepticus. She was hypotensive and bradycardic. Her electrocardiogram showed complete heart block. She was placed on transcutaneous pacer with no response. A transvenous pacemaker was placed before inserting a suitable permanent pacemaker for her size. This is the third case of complete heart block associated with Seckel syndrome and raises concern about the potential association.