Dorsal agenesis of the pancreas is a rare entity, with about 100 cases reported. It can be overlooked on ultrasound due to the non visualization of the body and tail of the pancreas. This is due to overlying gas in the stomach, which offers a poor acoustic window and obscures visualization. Renal agenesis and Mullerian duct anomalies are uncommon associations of dorsal agenesis of the pancreas because of the separate embryological origin of the pancreas and genitourinary organs. Here, we present a case of a 17-year-old patient who had dorsal agenesis of the pancreas, associated with unilateral renal agenesis, unicornuate uterus, and ectopic ovary. We describe the anomalies and discuss the radiological differential diagnosis and potential pitfalls. We provide a brief review of the literature with few radiological teaching points and possible genetic implications of the case.