Dioctophimosis is an endoparasitism in dogs caused by Dioctophyma renale, a nematode with an indirect life cycle and high zoonotic potential. Infection in dogs occurs through ingestion of transitional/paratenic hosts containing infective larvae. The preferred site of parasitism is the right kidney, although the parasite may also be ectopically localized in subcutaneous tissue, internal organs, peritoneum, and mediastinum. Since dogs are usually infected with a small number of parasites, the disease is asymptomatic, making the diagnosis of this disease in clinical veterinary practice rather difficult. In addition, as dogs are usually infected with a small number of parasites, the disease is asymptomatic, which makes the diagnosing of this disease in clinical veterinary practice difficult. The long prepatent period, lasting three to five months, makes it impossible to diagnose the disease in dogs younger than six months. Clinical suspicion of dioctophimosis in dogs is established on the basis of anamnestic/epizootiologic data and the clinical picture, while the final diagnosis is made ante mortem or post mortem on the basis of reliable diagnostic parameters and methods. The most practical application in routine diagnosis of canine dioctophimosis is the detection of D. renale eggs in urine sediment. When dioctophimosis is suspected in carnivores, the differential diagnosis should exclude urinary capillariosis, feline polycystic nephropathy, leptospirosis, renal fibrosis, and nephritis of various etiology. The outcome of treatment is uncertain and includes surgical removal of the affected kidney or drug therapy with avermectin derivatives. Prophylaxis consists of preventing contact of dogs with potential transitional hosts and controlling the feeding of their heat-untreated meat. Because of the potential risks that D. renale may have on the health of dog owners, education on the etiopathogenesis and means of occurrence, maintenance, and spread of this nematode is necessary.