Born in Brooklyn, New York City, the daughter of Dvora and Abraham Schneider (the name means tailor in German). Her family was Jewish, and had immigrated to the U.S. from Germany and Russia. Schneider studied piano before starting to perform as a singer in venues in New Englandand New York.
Between 1978 and 1984, she achieved success as a rock singer in Germany; her song "Rock'n' Roll Gypsy" reached the top 10 record charts and she received a Gold Record Award and shared the Goldene Europa Award with John Lennon. In 1980 she toured with the German rock legend Udo Lindenberg. She played one of the leads and the love interest of Eddie Wilson in the 1983 film Eddie and the Cruisers, which has since gained a huge cult following, especially in unusual places for an American film such as Nepal, Russia and especially Germany, where Schneider had name recognition.
In 1987, she began her acting career at the Theater des Westens in Berlin playing the Sally Bowles in the musical Cabaret. From 1995 to 1998, she performed as Norma Desmond in Sunset Boulevard by Andrew Lloyd Webber. She worked with Eberhard Schoener on the recording of the Short Opera (1996) and on the goodbye-song for the TV series Derrick. From 1999 to 2001, she performed as Eva Perón in the musical Evita at the Bad Hersfelder Festspiele. She received enormous acclaim for her one-woman performance pieces: A Walk on the Weill Side, A Voice and A Piano, and Transformations Ver.0.1. In 2006 she received the DIVA - German Entertainment Prize for her lifetime achievements.
In 2007 Helen Schneider returned to the recording business. She recorded three cd's with Edel Music. For "Dream a Little Dream" she received a golden CD award in jazz.