He may be best known for his plays, The Rez Sisters and Dry Lips Oughta Move to Kapuskasing, both of which won Dora Mavor Moore and Floyd S. Chalmers Awards.

His somewhat autobiographical novel, Kiss of the Fur Queen, has been acknowledged as one of the most powerful books about the north Cree experience (in fact, the human experience) and an extraordinary expression of love, loss, trial, victory, talent and heartbreak. A cornerstone of Canadian literature, Kiss of the Fur Queen is studied in universities around the world.

His plays have been performed in Toronto at the Royal Alexandra Theatre, in Scotland at the Edinburgh Festival, off-Broadway in New York, and in Tokyo. They have also been collected in anthologies that include Thornton Wilder, Tennessee Williams, Bertold Brecht, and Harold Pinter.

Highway has also written the first Cree language opera, The Journey of Pimooteewin. He is currently touring with a jazz/musical ensemble performing his own work, Songs in the Key of Cree.

He was awarded the Order of Canada in 1994.