The objective of this article is to review the literature on a very rare association such as patent urachus and a patent omphalo–mesenteric duct in children. Partial or total failure of the obliteration of urachus gives rise to various anomalies, which can be diagnosed both in childhood and in adulthood. The omphalo–mesenteric duct remnants are the most common anomalies of the gastro-intestinal tract, often asymptomatic. The association of patent urachus and a patent omphalo–mesenteric duct in children is very rare. A computer-assisted (PubMed and Google Scholar) search of the pediatric literature to identify all cases of patent urachus and a patent omphalo–mesenteric duct association was performed. We found only eleven cases of the association of patent urachus and a patent omphalo–mesenteric duct in the pediatric literature. All cases except one were males. When reported, all children affected were full term. The clinical sign reported was umbilical spillage. In all cases was reported a lack of healing of the umbilical stump with different clinical pictures, except two cases. Treatments reported were application of silver nitrate, antibiotic cream, povidone iodine, and application of salves and plasters. Various diagnostic examinations were performed. In none of these patients was the possible association with PU and POMD suspected, but it was evidenced only during the surgical excision. The surgical approach was laparotomy in all cases. The association of patent urachus and a patent omphalo–mesenteric duct in children is very rare approached in all cases by laparotomy. This review underlines the importance of evaluating any persisting umbilical lesions without delay when conventional pharmacological therapy fails.