Among the factors involved in male infertility, there is a rare morphology disorder called "globozoospermia" that is classified into total globozoospermia and partial globozoospermia (type I and type II, respectively). This syndrome is primarily characterised by the presence of round-headed spermatozoa with cytoskeleton defects around the nucleus and no acrosome. Current data support the negative correlation between globozoospermia and conventional intracytoplasmic sperm injection (ICSI) outcomes, revealing the need for the management of patients undergoing assisted reproduction technology (ART) through more effective treatment techniques. This review highlights the most important characteristics of globozoospermia such as sperm parameters, DNA/chromatin integrity and sperm DNA fragmentation (SDF), as well as genetic features based on the latest knowledge. Additionally, we looked into current progress on fertilisation potential and possible treatment strategies for patients presenting with globozoospermia.