Abdominal wall endometriosis (AWE) is one of the rarest forms of endometriosis. Little is known about differences between umbilical AWE (U-AWE) and non-umbilical AWE (non-U-AWE) patients. This retrospective cohort study included patients treated for AWE at tertiary endometriosis centre between 2012 and 2020. Patients were divided into two groups – umbilical AWE and non-umbilical AWE. We identified 14 U-AWE and 45 non-U-AWE patients who mostly had lesions in caesarean section scar (38, 64.4%), rarely at other locations (7, 11.9%). Infertility rates for U-AWE patients and non-U-AWE patients were 57.1% and 17.8%, respectively. Concurrent or previous peritoneal endometriosis was noted in 85.7% of U-AWE and 24.4% of non-U- AWE patients. In addition, U-AWE patients and non-UAWE patients significantly differed in following: parity, number of previous caesarean sections, lesion size, prevalence of concurrent or previous deep infiltrating endometriosis, bleeding from abdominal wall, cyclic pain, continuous pain. Infertility and pelvic endometriosis were more prevalent in U-AWE patients. Our data suggests that U-AWE may be a specific marker for a patient highly prone to pelvic endometriosis and subsequent infertility. Findings suggests that clinician should consider comprehensive evaluation of U-AWE patients.