SUMMARYInfertility affects approximately 15% of couples in reproductive age. Male infertility is estimated to represent about 20% of the etiologies. Among them, a rare type of teratozoospermia known as globozoospermia leads to disappointing pregnancy outcomes. Morphological, physiological and genetic aspects of this severe disorder have been described. We undertook a complete review of the available data on the reproductive outcomes in globozoospermic patients. To this end, a literature review in both English and French, over a 20-year time period using PubMed/Medline, ScienceDirect, and Scopus was performed. A total of 45 publications describing 172 attempts of treatment with assisted reproduction techniques (ICSI or IMSI with or without oocyte activation) were identified. We reviewed 28 deliveries and 34 children. However, for these patients, the fertilization rate after ICSI remained low. The present review suggests that oocyte activation (in particular with calcium ionophore) could improve the pregnancy rate significantly when dealing with globozoospermia. Once the exact pathogenesis of human globozoospermia is clearly identified, it is likely that other treatments such as recombinant phospholipase C zeta (PLC zeta, PLCf), which seems to be a promising biological tool, would be developed.