Unilateral ovarian absence (UOA) with fallopian tube absence is a rare condition. The true incidence is unknown. Here, we report a case of a woman with left tubal and ovarian absence, undergoing laparoscopy for an ipsilateral interstitial pregnancy (IP). The gestational mass was excided using the purse string technique to achieve better haemostasis. Probable etiologies are discussed and literature reviewed. According to our analysis the reproductive outcome of women affected by this rare anomaly is not compromised. Its impairment should be related to comorbidities or other unexplained causes. Â© Copyright 2014, CIC Edizioni Internazionali, Roma.