Butler was born in New Orleans, Louisiana. Following the devastation of Hurricane Katrina, Butler left New Orleans and relocated to Colorado, where he still resides.. He is known for his prodigious technique and his ability to play in many styles of music. Referred to by Dr. John as "the pride of New Orleans", Henry Butler is his generation's representative in the Crescent City's unique lineage of piano legends such as Professor Longhair, James Booker, Tuts Washington, Jelly Roll Morton, etc.

Butler has recorded for several record labels, including Impulse Records, Windham Hill, and Basin Street Records.

Blinded by glaucoma since birth, Butler has not stopped pursuing photography as a hobby; some of his photos have been shown in galleries in New Orleans.