I read this short story (or perhaps novelette) at least 40 years ago, in a collection. I realise now that the story might well be by John Varley. But I'm pretty sure it was not a pure Varley collection. It's only after I asked here this question that I started to associate frequent sex changes to Varley's "Eight Worlds" books, but there are so many of them...
So a young man (IIRC, XY genotype, but he had already been female, and has changed back) who lives with his mother knows he is a clone. For the first time his progenitor (of course the same genotype, but presently his older sister) comes to visit them on that planet, from the one from which his mother and himself came, and where she lived with one of their mother's friends. He knows there is something really bizarre in his family. Why did his mother leave her child with a friend, and move to another planet with a clone? But neither his mother nor his sister-progenitor would tell him the "big secret".
At some point clone-brother and progenitor-sister find themselves in a life-threatening situation. In these dire straits, the sister tells him all.
The woman she was living with is really their common mother. The supposed "mother" of the young man is in fact their common father.
They survive but I don't remember the details of the end of the story.