He was born as William James Dixon, in Vicksburg, Mississippi. He was a producer for Chess and Checker Records in Chicago and is considered one of the key figures in the creation of Chicago blues. He worked with Chuck Berry, Muddy Waters, Howlin' Wolf, Otis Rush, Bo Diddley, Little Walter, Sonny Boy Williamson, Koko Taylor, Little Milton, Eddie Boyd, Jimmy Witherspoon, Lowell Fulson, Willie Mabon, Memphis Slim, Washboard Sam, Jimmy Rogers, and others.

He had a colorful life. In his teens he had many scrapes with the law, and decided to hitchhike his way to Chicago. A giant of a man, he took up boxing, and was so successful as to win the Golden Gloves heavyweight title in 1936. His progress in learning to play the bass was halted when he resisted the World War II draft, and was imprisoned for ten months. After the war, he re-united with his bass playing tutor, Baby Doo Caston, forming the Big Three Trio, who went on to record for Columbia Records. Dixon subsequently signed for Chess Records as a recording artist, but by 1951 he was a full time employee of the label. His relationship with them was sometimes strained, although his spell there covered the years from 1948 to the early 1960s. During this time his output, and influence was prodigious.

Indeed, he once claimed "I am the blues." This may seem a little arrogant, but there is no doubt that he was one of the major influences on the genre, through his original and varied songwriting, live performances, recording, and copious production work. He later recorded on Bluesville Records.

His double bass playing was of a high standard. He appears on many of Chuck Berry's early recordings, further proving his linkage between the blues and the birth of rock 'n' roll.

Dixon's genius as a songwriter lay in refurbishing archaic Southern motifs, in contemporary arrangements. This produced songs with the backbone of the blues, and the agility of pop music. British R&B bands of the 1960s constantly drew on the Dixon songbook for inspiration.

In addition, as his songwriting and production work started to take a backseat, his organisational ability was utilised, putting together all-star, Chicago based blues ensembles for work in Europe.

In "New York Dolls: All Dolled Up," David Johansen tells a story about how Dixon used to offer meals to songwriters newly in Chicago from the Delta in exchange to rights for their songs. Johansen claims Hoochie Coochie Man was one such song and called Dixon "The Vampire of the Blues."

His health deteriorated in the 1970s and 1980s, due to long-term diabetes, and eventually his leg had to be amputated. Willie Dixon died in 1992 and was posthumously inducted into the Rock and Roll Hall of Fame in 1994. As the songlist below demonstrates, his work was covered by a varied range of artists, from the blues, to modern day rock music practitioners.

Willie Dixon died of heart failure in Burbank, California in 1992 and was buried in the Burr Oak Cemetery in Alsip, Illinois.