AimPrimary splenic pregnancy is a rare form of extratubal ectopic pregnancy, frequently associated with life‐threatening abdominal bleeding, with only 51 cases reported in the worldwide literature. The aim of this work, through the proposal of its own index case, is to identify the fundamental steps of the diagnostic process, crucial to reduce the mortality rate, while evaluating the available therapeutic options, through a review of the literature, particularly the more conservative ones, to avoid splenectomy.MethodsWe present the case of a 22‐year‐old woman, showing her diagnostic‐therapeutic pathway. We searched Pubmed and other sources for all publications in English and all available non‐English literature, including historical publications, on this rare topic, to identify the best treatment for these patients. We classified each article by clinical onset, diagnostic and therapeutic strategy, and histological findings, if available.ResultsWe reviewed all the 43 cases available in the English‐language literature (plus another paper in German), finding that 72.7% of patients presented in an emergency setting. Seventy‐five percent of patients required splenectomy, 6.8% received pharmacological‐only therapy, 11.3% received arterial embolization before definitive treatment. The other ones received non‐radical surgical treatment.ConclusionsPrimary splenic pregnancy presents challenging diagnostic and therapeutic features. Treatment options (pharmacological, interventional, and/or surgical) should be tailored based on the clinical presentation and hemodynamic status of the patient. Our work would like to stimulate the validation of evidence‐based therapeutic strategies validated by clinical experience.