Aim: Presentation of an abdominoscrotal hydrocele case with a very rare association to multiple anomalies and separate abdominal and inguinoscrotal sacs. Case Report: A 21-year-old male patient presented with left-sided abdominoscrotal hydrocele, impalpable right testis, hydrocephalus and cerebral and musculoskeletal deformities. On abdominal ultrasonography, the abdominal and inguinoscrotal components were seen separated by a septum that was confirmed on surgery. Extended inguinal incision was used for repair and the post-operative course was uneventful. Discussion: Abdominoscrotal hydrocele is a rare entity and its simultaneous association to multiple anomalies, like in the current case, is a rarer finding. Non-intercommunication state is another very rare criteria of abdominoscrotal hydrocele which was clinically, sonographically and surgically demonstrated in this case. Extended inguinal incision was indicated and enough to deal with both abdominal and scrotal components. Conclusion: Abdominoscrotal hydrocele is mostly intercommunicating, but it is exceptionally non-intercommunicating and associated to multiple anomalies. Surgical excision via an extended inguinal incision may be indicated for voluminous cases and those with a solitary testis.