We describe here an autopsy case presenting bilateral cryptorchidism, left pelvic kidney and intestinal malrotation, evoking a “reverse rotation” with hypoplasia of the cecum. Furthermore, the colon showed alternate bands of stenosis and dilatation. This association has not yet been described, and is discussed in the light of the embryological events of kidney, gut and gonad development. Two hypotheses can explain gut stenoses (vascular consequences of the malrotation and type Hirschprung aganglionic segments).